crystallins

Summary

Summary: A heterogeneous family of water-soluble structural proteins found in cells of the vertebrate lens. The presence of these proteins accounts for the transparency of the lens. The family is composed of four major groups, alpha, beta, gamma, and delta, and several minor groups, which are classed on the basis of size, charge, immunological properties, and vertebrate source. Alpha, beta, and delta crystallins occur in avian and reptilian lenses, while alpha, beta, and gamma crystallins occur in all other lenses.

Top Publications

  1. pmc Mutation analysis of CRYAA, CRYGC, and CRYGD associated with autosomal dominant congenital cataract in Brazilian families
    Alessandro Santana
    Cataract Service, Department of Ophthalmology, Faculty of Medical Sciences, Irmandade da Santa Casa de Misericordia de Sao Paulo, Sao Paulo, Brazil
    Mol Vis 15:793-800. 2009
  2. ncbi Comprehensive mutational screening in a cohort of Danish families with hereditary congenital cataract
    Lars Hansen
    Wilhelm Johannsen Centre for Functional Genome Research, Institute of Cellular and Molecular Medicine, University of Copenhagen, Copenhagen, Denmark
    Invest Ophthalmol Vis Sci 50:3291-303. 2009
  3. pmc Congenital cataracts and their molecular genetics
    J Fielding Hejtmancik
    MOGS OGVFB NEI NIH, Building 10, Bethesda, MD 20892, USA
    Semin Cell Dev Biol 19:134-49. 2008
  4. pmc Lens aging: effects of crystallins
    K Krishna Sharma
    Department of Ophthalmology, University of Missouri Columbia, 1 Hospital Drive, Columbia, MO 65212, USA
    Biochim Biophys Acta 1790:1095-108. 2009
  5. ncbi The cellular basis of corneal transparency: evidence for 'corneal crystallins'
    J V Jester
    Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
    J Cell Sci 112:613-22. 1999
  6. pmc The retinal proteome in experimental diabetic retinopathy: up-regulation of crystallins and reversal by systemic and periocular insulin
    Patrice E Fort
    Department of Ophthalmology, Penn State College of Medicine, Hershey, Pennsylvania 17033, USA
    Mol Cell Proteomics 8:767-79. 2009
  7. pmc Regulation of gene expression by Pax6 in ocular cells: a case of tissue-preferred expression of crystallins in lens
    Ales Cvekl
    The Department of Ophthalmology, Albert Einstein College of Medicine, Bronx, NY 10461, USA
    Int J Dev Biol 48:829-44. 2004
  8. ncbi Cytoskeletal competence requires protein chaperones
    Roy Quinlan
    Department of Biological Sciences, South Road, Durham DH1 3LE, UK
    Prog Mol Subcell Biol 28:219-33. 2002
  9. ncbi Genomic analyses reveal global functional alterations that promote tumor growth and novel tumor suppressor genes in natural killer-cell malignancies
    J Iqbal
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USA
    Leukemia 23:1139-51. 2009
  10. pmc A proteome map of the zebrafish (Danio rerio) lens reveals similarities between zebrafish and mammalian crystallin expression
    Mason Posner
    Department of Biology, Ashland University, Ashland, OH 2Department of Chemistry, Ashland University, Ashland, OH 44805, USA
    Mol Vis 14:806-14. 2008

Detail Information

Publications304 found, 100 shown here

  1. pmc Mutation analysis of CRYAA, CRYGC, and CRYGD associated with autosomal dominant congenital cataract in Brazilian families
    Alessandro Santana
    Cataract Service, Department of Ophthalmology, Faculty of Medical Sciences, Irmandade da Santa Casa de Misericordia de Sao Paulo, Sao Paulo, Brazil
    Mol Vis 15:793-800. 2009
    ..The purpose of this study was to investigate mutations in alphaA-crystallin (CRYAA), gammaC-crystallin (CRYGC), and gammaD-crystallin (CRYGD) in Brazilian families with nuclear and lamellar autosomal dominant congenital cataract...
  2. ncbi Comprehensive mutational screening in a cohort of Danish families with hereditary congenital cataract
    Lars Hansen
    Wilhelm Johannsen Centre for Functional Genome Research, Institute of Cellular and Molecular Medicine, University of Copenhagen, Copenhagen, Denmark
    Invest Ophthalmol Vis Sci 50:3291-303. 2009
    ..Seven families have been published previously, and the data of the remaining 21 families are presented together with an overview of the results in all families...
  3. pmc Congenital cataracts and their molecular genetics
    J Fielding Hejtmancik
    MOGS OGVFB NEI NIH, Building 10, Bethesda, MD 20892, USA
    Semin Cell Dev Biol 19:134-49. 2008
    ..The increasing availability of more detailed information about these proteins and their functions and is making it possible to understand the pathophysiology of cataracts and the biology of the lens in general...
  4. pmc Lens aging: effects of crystallins
    K Krishna Sharma
    Department of Ophthalmology, University of Missouri Columbia, 1 Hospital Drive, Columbia, MO 65212, USA
    Biochim Biophys Acta 1790:1095-108. 2009
    ..The major proteins in the lens--alpha, beta, and gamma-crystallins--are constantly subjected to age-related changes such as oxidation, deamidation, truncation, glycation, and ..
  5. ncbi The cellular basis of corneal transparency: evidence for 'corneal crystallins'
    J V Jester
    Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
    J Cell Sci 112:613-22. 1999
    ..of TKT and ALDH1 contribute to corneal transparency in the rabbit at the cellular level, reminiscent of enzyme-crystallins in the lens...
  6. pmc The retinal proteome in experimental diabetic retinopathy: up-regulation of crystallins and reversal by systemic and periocular insulin
    Patrice E Fort
    Department of Ophthalmology, Penn State College of Medicine, Hershey, Pennsylvania 17033, USA
    Mol Cell Proteomics 8:767-79. 2009
    ..A detailed time course study during diabetes showed differential up-regulation of the different isoforms of the crystallins superfamily...
  7. pmc Regulation of gene expression by Pax6 in ocular cells: a case of tissue-preferred expression of crystallins in lens
    Ales Cvekl
    The Department of Ophthalmology, Albert Einstein College of Medicine, Bronx, NY 10461, USA
    Int J Dev Biol 48:829-44. 2004
    ..of lens is characterized by lens-preferred expression and accumulation of water-soluble proteins, crystallins. Crystallins are required for light transparency, refraction and maintenance of lens integrity...
  8. ncbi Cytoskeletal competence requires protein chaperones
    Roy Quinlan
    Department of Biological Sciences, South Road, Durham DH1 3LE, UK
    Prog Mol Subcell Biol 28:219-33. 2002
  9. ncbi Genomic analyses reveal global functional alterations that promote tumor growth and novel tumor suppressor genes in natural killer-cell malignancies
    J Iqbal
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USA
    Leukemia 23:1139-51. 2009
    ..ATG5, an essential gene for autophagy and AIM1, a gene implicated in melanoma, may also participate in the functional abnormalities...
  10. pmc A proteome map of the zebrafish (Danio rerio) lens reveals similarities between zebrafish and mammalian crystallin expression
    Mason Posner
    Department of Biology, Ashland University, Ashland, OH 2Department of Chemistry, Ashland University, Ashland, OH 44805, USA
    Mol Vis 14:806-14. 2008
    ..To characterize the crystallin content of the zebrafish lens using two-dimensional gel electrophoresis (2-DE). These data will facilitate future investigations of vertebrate lens development, function, and disease...
  11. pmc Identification of CRYM as a candidate responsible for nonsyndromic deafness, through cDNA microarray analysis of human cochlear and vestibular tissues
    Satoko Abe
    Laboratory of Molecular Medicine, Human Genome Center, Institute of Medical Science, The University of Tokyo, Tokyo, Japan
    Am J Hum Genet 72:73-82. 2003
    ..Our results strongly implicate CRYM in normal auditory function and identify it as one of the genes that can be responsible for nonsyndromic deafness...
  12. ncbi sHSP in the eye lens: crystallin mutations, cataract and proteostasis
    Alice R Clark
    Department of Biological Sciences, Crystallography, Institute of Structural and Molecular Biology, Birkbeck College, Malet Street, London WC1E 7HX, UK
    Int J Biochem Cell Biol 44:1687-97. 2012
    ..crystallin mutations are a common cause of childhood cataract, whereas miscellaneous changes to the long-lived crystallins cause age-related cataract, the most common cause of blindness worldwide...
  13. ncbi Eye evolution: lens and cornea as an upgrade of animal visual system
    Kristyna Jonasova
    Department of Transcriptional Regulation, Institute of Molecular Genetics, Academy of Sciences of the Czech Republic, Prague 4, Czech Republic
    Semin Cell Dev Biol 19:71-81. 2008
    ..addition to having an ancient role in prototypical eye formation Pax transcription factors were convergently recruited for regulation of structurally diverse crystallins and genes affecting morphogenesis of various lens-containing eyes.
  14. pmc Mutation analysis of 12 genes in Chinese families with congenital cataracts
    Wenmin Sun
    State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat Sen University, Guangzhou, China
    Mol Vis 17:2197-206. 2011
    ..To identify mutations in 12 genes in Chinese families with congenital cataracts...
  15. ncbi Simultaneous racemization and isomerization at specific aspartic acid residues in alpha B-crystallin from the aged human lens
    N Fujii
    Discovery Research Division, Takeda Chemical Industries, Ltd, Ibaraki, Japan
    Biochim Biophys Acta 1204:157-63. 1994
    ..We concluded that racemization, isomerization, and oxidation of alpha B-crystallin occur spontaneously in the aging process...
  16. ncbi Decoding the molecular design principles underlying Ca(2+) binding to βγ-crystallin motifs
    Amita Mishra
    Centre for Cellular and Molecular Biology, Council of Scientific and Industrial Research, Hyderabad 500007, India
    J Mol Biol 415:75-91. 2012
    ..Decoding such design schemes would aid in our understanding of the functional themes underlying differential Ca(2)(+) binding and in predicting these in emerging sequence information...
  17. ncbi Characterization of the G91del CRYBA1/3-crystallin protein: a cause of human inherited cataract
    M A Reddy
    Department of Molecular Genetics, Institute of Ophthalmology, 11 43 Bath Street, London EC1V 9EL, UK
    Hum Mol Genet 13:945-53. 2004
    ..b>Crystallins are abundantly expressed in the developing human lens and represent excellent candidate genes for inherited ..
  18. ncbi A deletion mutation in the betaA1/A3 crystallin gene ( CRYBA1/A3) is associated with autosomal dominant congenital nuclear cataract in a Chinese family
    Yanhua Qi
    Department of Ophthalmology, The Second Affiliated Hospital, Haerbin Medical University, Haerbin, 150086 Heilongjiang, P R China
    Hum Genet 114:192-7. 2004
    ..It highlights the physiological importance of crystallin and supports the role of CRYBA1/A3 in human cataracts formation...
  19. ncbi Transgene overexpression of alphaB crystallin confers simultaneous protection against cardiomyocyte apoptosis and necrosis during myocardial ischemia and reperfusion
    P S Ray
    Cardiovascular Research Center, Department of Surgery, University of Connecticut Health Center, Farmington, Connecticut 06030 1110, USA
    FASEB J 15:393-402. 2001
    ..Transgene overexpression of alphaB crystallin was therefore successful in diminishing the independent contributory effects of both necrosis and apoptosis on I/R-induced cell death...
  20. pmc The ageing lens and cataract: a model of normal and pathological ageing
    R Michael
    Institut Universitari Barraquer, Universitat Autonoma de Barcelona, Laforja 88, 08021 Barcelona, Spain
    Philos Trans R Soc Lond B Biol Sci 366:1278-92. 2011
    ..Unlike nuclear cataract, progression is intermittent, stepwise and non-uniform...
  21. ncbi Ageing and vision: structure, stability and function of lens crystallins
    Hans Bloemendal
    Department of Biochemistry, University of Nijmegen, 6500HB, The Netherlands
    Prog Biophys Mol Biol 86:407-85. 2004
    The alpha-, beta- and gamma-crystallins are the major protein components of the vertebrate eye lens, alpha-crystallin as a molecular chaperone as well as a structural protein, beta- and gamma-crystallins as structural proteins...
  22. ncbi Crystallins of the beta/gamma-superfamily mimic the effects of lens injury and promote axon regeneration
    Dietmar Fischer
    Department of Experimental Neurology, Medical School, University of Ulm, Albert Einstein Allee 11, D89081 Ulm, Germany
    Mol Cell Neurosci 37:471-9. 2008
    ..The present work reveals that certain purified lens proteins (crystallins) cause the effects of LI...
  23. ncbi Muscle develops a specific form of small heat shock protein complex composed of MKBP/HSPB2 and HSPB3 during myogenic differentiation
    Y Sugiyama
    Department of Molecular Biology, Yokohama City University School of Medicine, Kanazawa Ku, Yokohama 236 0004, Japan
    J Biol Chem 275:1095-104. 2000
    ..Taken together, the results suggest that muscle cells develop two kinds of stress response systems composed of diverged sHSP members, and that these systems work independently in muscle maintenance and differentiation...
  24. pmc AIM1, a novel non-lens member of the betagamma-crystallin superfamily, is associated with the control of tumorigenicity in human malignant melanoma
    M E Ray
    Laboratory of Cancer Genetics, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 94:3229-34. 1997
    ..AIM1 is a good candidate for the putative suppressor of malignant melanoma on chromosome 6, possibly exerting its effects through interactions with the cytoskeleton...
  25. ncbi Molecular characteristics of inherited congenital cataracts
    Bingyu Huang
    Medical Genetics Laboratory, Department of Obstetrics and Gynecology, Second Teaching Hospital, Jilin University, 218 Zhiqiang, Changchun, 130041, China
    Eur J Med Genet 53:347-57. 2010
    ..Inherited congenital cataracts have been associated with mutations in specific genes, including those of crystallins, gap junction proteins, membrane transport and channel proteins, the cytoskeleton, and growth and transcription ..
  26. ncbi A novel mutation in AlphaA-crystallin (CRYAA) caused autosomal dominant congenital cataract in a large Chinese family
    Feng Gu
    Department of Genetics, National Research Institute for Family Planning, Beijing, China
    Hum Mutat 29:769. 2008
    ..R116H). To understand the molecular basis of cataract formation, mutant and wild-type alphaA-crystallins were expressed in E. coli...
  27. pmc Mutation G61C in the CRYGD gene causing autosomal dominant congenital coralliform cataracts
    Feifeng Li
    Graduate School, Peking Union Medical College, Beijing, China
    Mol Vis 14:378-86. 2008
    ..We sought to identify the genetic defect in a four-generation Chinese family with autosomal dominant congenital coralliform cataracts and demonstrate the functional analysis of a candidate gene in the family...
  28. ncbi Cloning, expression, and chaperone-like activity of human alphaA-crystallin
    U P Andley
    Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 271:31973-80. 1996
    ....
  29. pmc Vitreoretinal influences on lens function and cataract
    David C Beebe
    Department of Ophthalmology and Visual Sciences, Washington University, Saint Louis, MO 63110, USA
    Philos Trans R Soc Lond B Biol Sci 366:1293-300. 2011
    ..Oxygen reaches the lens nucleus, increasing protein oxidation and aggregation and leading to nuclear cataract. We suggest that maintaining low pO(2) around the lens should prevent the formation of nuclear cataracts...
  30. ncbi Lens proteomics: analysis of rat crystallins when lenses are exposed to dexamethasone
    Lin Wang
    Harbin Medical University, Harbin, China
    Mol Biosyst 8:888-901. 2012
    To identify glucocorticoid induced cataract (GIC)-specific modified crystallins and related changes, we analyzed rat crystallins and related changes in lenses exposed to dexamethasone (Dex)...
  31. pmc βA3/A1-crystallin is required for proper astrocyte template formation and vascular remodeling in the retina
    Debasish Sinha
    The Wilmer Eye Institute, The Johns Hopkins University School of Medicine, 400 North Broadway, Baltimore, MD, 21231, USA
    Transgenic Res 21:1033-42. 2012
    ....
  32. ncbi Lens gene expression analysis reveals downregulation of the anti-apoptotic chaperone alphaA-crystallin during cavefish eye degeneration
    Allen G Strickler
    Department of Biology, University of Maryland, College Park, MD 20742, USA
    Dev Genes Evol 217:771-82. 2007
    ..The genes encoding the lens intrinsic membrane proteins MIP and MP19 and the divergent betaB1- and gammaM2-crystallins are expressed during cavefish lens development, although their levels are reduced because of a smaller lens, ..
  33. ncbi Differential regulation of gamma-crystallin genes during mouse lens development
    M Murer-Orlando
    Dev Biol 119:260-7. 1987
    ..This complex pattern of gene regulation presumably accounts for one of the mechanisms determining the spatial distribution of different gamma-crystallins within the lens.
  34. ncbi Identification and characterization of the enzymatic activity of zeta-crystallin from guinea pig lens. A novel NADPH:quinone oxidoreductase
    P V Rao
    Laboratory of Mechanisms of Ocular Diseases, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892
    J Biol Chem 267:96-102. 1992
    ..As with several other lens crystallins of limited phylogenetic distribution, zeta-crystallin has been characterized as an "enzyme/crystallin" based on ..
  35. ncbi Bmp signaling is required for development of primary lens fiber cells
    Sonya C Faber
    Division of Developmental Biology and Department of Ophthalmology, Children s Hospital Research Foundation, 3333 Burnet Avenue Cincinnati, OH 45229, USA
    Development 129:3727-37. 2002
    ..We conclude that Bmp signaling is required for primary lens fiber cell differentiation and, given the asymmetry of the differentiation inhibition, that distinct differentiation stimuli may be active in different quadrants of the eye...
  36. ncbi Phosphorylation-induced change of the oligomerization state of alpha B-crystallin
    H Ito
    Department of Biochemistry, Institute for Developmental Research, Aichi Human Service Center, Kasugai, Aichi 480 0392, Japan
    J Biol Chem 276:5346-52. 2001
    ..From these observations, it is suggested that phosphorylation of alphaB-crystallin causes dissociation of large oligomers to smaller sizes molecules and reduction of chaperone-like activity, like in the case of HSP27...
  37. ncbi X-ray analysis of the eye lens protein gamma-II crystallin at 1.9 A resolution
    G Wistow
    J Mol Biol 170:175-202. 1983
    ..Crystal lattice interactions are described which may be similar to those occurring in vivo in the lens between crystallins. Seven cysteine residues have been identified in the structure and these may have a role in the thermodynamic ..
  38. ncbi Colloidal characterization and thermodynamic stability of binary eye lens protein mixtures
    N Dorsaz
    Institut Romand de Recherche Numérique en Physique des Matériaux, IRRMA, Ecole Polytechnique Federale de Lausanne EPFL, CH 1015 Lausanne, Switzerland
    J Phys Chem B 113:1693-709. 2009
    ..The relevance of these mechanisms as possible sources of the alteration of the spatial distribution of the lens proteins encountered in cataract disease is discussed...
  39. ncbi Sulfur in human crystallins
    Durga Srikanthan
    Department of Crystallography, Birkbeck College, University of London, Malet Street, London WC1E 7HX, UK
    Exp Eye Res 79:823-31. 2004
    Molecular models of human gamma-crystallins and the 'alpha-crystallin domain' of human alphaA-crystallin have been built based on available related X-ray crystal structures...
  40. ncbi Up-regulation of crystallins is involved in the neuroprotective effect of wolfberry on survival of retinal ganglion cells in rat ocular hypertension model
    Kin Chiu
    Laboratory of Neurodegenerative Diseases, Department of Anatomy, LKS Faculty of Medicine, The University of Hong Kong, Pokfulam, Hong Kong SAR, China
    J Cell Biochem 110:311-20. 2010
    ..We demonstrated that neuroprotective effect of-wolfberry extract-LBP on the survival of RGCs may be mediated via direct up-regulation of neuronal survival signal betaB2-crystallin...
  41. ncbi Conversion of arginine into ornithine by advanced glycation in senescent human collagen and lens crystallins
    David R Sell
    Institute of Pathology, School of Medicine, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 279:54173-84. 2004
    ..of ornithine, the nonprotein amino acid, and decarbamidation product of arginine in acid hydrolysates of lens crystallins and skin collagen led us to investigate its source and mechanism of formation...
  42. ncbi A comprehensive analysis of the expression of crystallins in mouse retina
    Jinghua Xi
    Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, Missouri 63110, USA
    Mol Vis 9:410-9. 2003
    b>Crystallins are expressed at high levels in lens fiber cells. Recent studies have revealed that several members of the alpha, beta, and gamma-crystallin family are also distributed in many non-lens tissues, though at lower levels...
  43. ncbi Connexin 46 and connexin 50 in selenite cataract
    C R Fleschner
    A T Still University of Health Sciences, Kirksville College of Osteopathic Medicine, MO 63501 1443, USA
    Ophthalmic Res 38:24-8. 2006
    ....
  44. pmc Tight binding of proteins to membranes from older human cells
    Roger J W Truscott
    Save Sight Institute, Sydney University, Sydney, NSW, Australia
    Age (Dordr) 33:543-54. 2011
    ..A majority of lens beta crystallins and all gamma crystallins became increasingly membrane bound with age, however, the chaperone proteins alpha A ..
  45. ncbi Intense light exposure changes the crystallin content in retina
    Hirokazu Sakaguchi
    Exp Eye Res 76:131-3. 2003
    ..Following light exposure, anti-alphaB-crystallin immunoreactivity was increased in rod outer segments and retinal pigment epithelium. These findings support a possible role for crystallins in protecting photoreceptors from light damage.
  46. pmc Pax6 is essential for lens fiber cell differentiation
    Ohad Shaham
    Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
    Development 136:2567-78. 2009
    ..This study reveals that Pax6 is pivotal for initiation of the lens fiber differentiation program in the mammalian eye...
  47. ncbi Energetics of domain-domain interactions and entropy driven association of beta-crystallins
    Y V Sergeev
    National Eye Institute, National Institutes of Health, Bethesda, Maryland 20982, USA
    Biochemistry 43:415-24. 2004
    Beta-crystallins are major protein constituents of the mammalian lens, where their stability and association into higher order complexes are critical for lens clarity and refraction...
  48. pmc Unfolding crystallins: the destabilizing role of a beta-hairpin cysteine in betaB2-crystallin by simulation and experiment
    James T MacDonald
    School of Crystallography, Birkbeck College, Malet Street, London, WC1E 7HX, UK
    Protein Sci 14:1282-92. 2005
    The thermodynamic and kinetic stabilities of the eye lens family of betagamma-crystallins are important factors in the etiology of senile cataract...
  49. pmc Age-related changes in human crystallins determined from comparative analysis of post-translational modifications in young and aged lens: does deamidation contribute to crystallin insolubility?
    P A Wilmarth
    Department of Integrative Biosciences, School of Dentistry, Oregon Health and Science University, 611 South West Campus Drive, Portland, Oregon 97239, USA
    J Proteome Res 5:2554-66. 2006
    ..In total, 491 modification sites in lens proteins were identified. There were 155 in vivo PTM sites in crystallins: 77 previously reported sites and 78 newly detected PTM sites...
  50. ncbi Identification and characterization of the gene encoding a new member of the alpha-crystallin/small hsp family, closely linked to the alphaB-crystallin gene in a head-to-head manner
    A Iwaki
    Institute of Genetic Information, Kyushu University 18, Fukuoka, 812 82, Japan
    Genomics 45:386-94. 1997
    ..The two related genes are arranged in a head-to-head manner with an intergenic sequence of less than 1 kb, raising a possibility of shared regulatory elements for their expression...
  51. pmc Glycation by ascorbic acid oxidation products leads to the aggregation of lens proteins
    Mikhail Linetsky
    Mason Eye Institute, University of Missouri Columbia 65201, USA
    Biochim Biophys Acta 1782:22-34. 2008
    ..This aggregation was likely due to the glycation of lens crystallins because [U-(14)C] ascorbate was incorporated into the aggregate fraction and because NaCNBH(3), which reduces ..
  52. ncbi Characterization of covalent multimers of crystallins in aging human lenses
    Om P Srivastava
    Department of Physiological Optics, University of Alabama at Birmingham, Birmingham, Alabama 35294, USA
    J Biol Chem 279:10901-9. 2004
    ..e. the first type composed of fragments of eight different crystallins (i.e...
  53. pmc Vitamin C mediates chemical aging of lens crystallins by the Maillard reaction in a humanized mouse model
    Xingjun Fan
    Department of Pathology, Case Western Reserve University, 2103 Cornell Road, Cleveland, OH 44106 7288, USA
    Proc Natl Acad Sci U S A 103:16912-7. 2006
    ..with progressive oxidation, fragmentation, cross-linking, insolubilization, and yellow pigmentation of lens crystallins. We hypothesized that the Maillard reaction, which leads browning and aroma development during the baking of ..
  54. ncbi Mechanisms involved in the protection of UV-induced protein inactivation by the corneal crystallin ALDH3A1
    Tia Estey
    Center for Pharmaceutical Biotechnology and Molecular Toxicology and Environmental Health Sciences Program, Department of Pharmaceutical Sciences, University of Colorado Health Sciences Center, Denver, Colorado 80262, USA
    J Biol Chem 282:4382-92. 2007
    ..This study provides for the first time mechanistic evidence supporting the structural role of the corneal crystallin ALDH3A1 as a UV-absorbing constituent of the cornea...
  55. pmc A novel non-lens betagamma-crystallin and trefoil factor complex from amphibian skin and its functional implications
    Shu Bai Liu
    Biotoxin Units, Key Laboratory of Animal Models and Human Disease Mechanisms, Kunming Institute of Zoology, The Chinese Academy of Sciences, Kunming, Yunnan, China
    PLoS ONE 3:e1770. 2008
    In vertebrates, non-lens betagamma-crystallins are widely expressed in various tissues, but their functions are unknown...
  56. pmc Mutation in the βA3/A1-crystallin gene impairs phagosome degradation in the retinal pigmented epithelium of the rat
    J Samuel Zigler
    The Wilmer Eye Institute, The Johns Hopkins University School of Medicine, 600 N Wolfe Street, Baltimore, MD 21287, USA
    J Cell Sci 124:523-31. 2011
    ..A3/A1-crystallin is a novel lysosomal protein in RPE, essential for degradation of phagocytosed material...
  57. ncbi Genetics of crystallins: cataract and beyond
    Jochen Graw
    Helmholtz Center Munich, German Research Center for Environmental Health, Institute of Developmental Genetics, D 85764 Neuherberg, Germany
    Exp Eye Res 88:173-89. 2009
    The crystallins were discovered more than 100 years ago by Mörner (1893. Untersuchungen der Proteinsubstanzen in den lichtbrechenden Medien des Auges. Z. Physiol. Chem...
  58. ncbi Review: A case for corneal crystallins
    J Piatigorsky
    Laboratory of Molecular and Developmental Biology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892 2730, USA
    J Ocul Pharmacol Ther 16:173-80. 2000
    It is established that the diverse, multifunctional crystallins are responsible for the optical properties of the cellular, transparent lens of the complex eyes of vertebrates and invertebrates...
  59. pmc Alpha-crystallin-type heat shock proteins: socializing minichaperones in the context of a multichaperone network
    Franz Narberhaus
    Institut fur Mikrobiologie, Eidgenossische Technische Hochschule, CH 8092 Zurich, Switzerland
    Microbiol Mol Biol Rev 66:64-93; table of contents. 2002
    Alpha-crystallins were originally recognized as proteins contributing to the transparency of the mammalian eye lens. Subsequently, they have been found in many, but not all, members of the Archaea, Bacteria, and Eucarya...
  60. pmc Formation of amyloid fibrils in vitro by human gammaD-crystallin and its isolated domains
    Katerina Papanikolopoulou
    Department of Materials Science and Technology, University of Crete, Heraklion, Crete, Greece
    Mol Vis 14:81-9. 2008
    ..Previous studies have shown that bovine crystallins form amyloid fibers under denaturing conditions and amyloid fibers accumulate in the lens of mice carrying ..
  61. ncbi Genealogy of the alpha-crystallin--small heat-shock protein superfamily
    W W de Jong
    Department of Biochemistry, University of Nijmegen, The Netherlands
    Int J Biol Macromol 22:151-62. 1998
    ..It is suggested that during eukaryote evolution, animal and non-animal alpha-Hsps originated from different ancestral gene copies. Repeated gene duplications gave rise to the multiple alpha-Hsps present in most organisms...
  62. pmc Dual roles for Pax-6: a transcriptional repressor of lens fiber cell-specific beta-crystallin genes
    M K Duncan
    Department of Biological Sciences, The University of Delaware, Newark, Delaware 19716, USA
    Mol Cell Biol 18:5579-86. 1998
    ....
  63. ncbi Cleavage of the C-terminal serine of human alphaA-crystallin produces alphaA1-172 with increased chaperone activity and oligomeric size
    Atya Aziz
    Department of Biochemistry and Molecular Biology, University of Arkansas for Medical Sciences, Little Rock, Arkansas 72205, USA
    Biochemistry 46:2510-9. 2007
    ..Various truncated forms of human alphaA-crystallins were prepared by site-directed mutagenesis...
  64. ncbi CRYGD gene analysis in a family with autosomal dominant congenital cataract: evidence for molecular homogeneity and intrafamilial clinical heterogeneity in aculeiform cataract
    Juan Carlos Zenteno
    Department of Genetics, Instituto de Oftalmología Conde de Valenciana, Mexico City, Mexico
    Mol Vis 11:438-42. 2005
    ..To present a previously unreported four generation affected Mexican pedigree with congenital hereditary aculeiform cataract caused by a mutation in the gammaD-crystallin (CRYGD) gene...
  65. ncbi A novel deletion variant of gammaD-crystallin responsible for congenital nuclear cataract
    Li Yun Zhang
    Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong, China
    Mol Vis 13:2096-104. 2007
    ..To investigate a novel deletion variant of gammaD-crystallin (CRYGD) identified in a Chinese family with nuclear congenital cataract...
  66. ncbi AlphaB-crystallin in lens development and muscle integrity: a gene knockout approach
    J P Brady
    National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892 2730, USA
    Invest Ophthalmol Vis Sci 42:2924-34. 2001
    ..To study the role of alphaB-crystallin (alphaB) in the developing lens and its importance in lens structure and function...
  67. ncbi R120G alphaB-crystallin promotes the unfolding of reduced alpha-lactalbumin and is inherently unstable
    Teresa M Treweek
    Department of Chemistry, University of Wollongong, NSW, Australia
    FEBS J 272:711-24. 2005
    ..These characteristics may explain the involvement of R120G alphaB-crystallin with human disease states...
  68. ncbi Redox regulation in the lens
    Marjorie F Lou
    Redox Biology Center, University of Nebraska Lincoln, Lincoln, NE 68583, USA
    Prog Retin Eye Res 22:657-82. 2003
    ..These two enzymes may work synergistically to regulate and repair thiols in lens proteins and enzymes, keeping a balanced redox potential to maintain the function of the lens...
  69. pmc Age-dependent deamidation of lifelong proteins in the human lens
    Peter G Hains
    Save Sight Institute, University of Sydney, Sydney, NSW, Australia
    Invest Ophthalmol Vis Sci 51:3107-14. 2010
    Deamidation is a common posttranslational modification in human lens crystallins and may be a key factor in the age-related denaturation of such lifelong proteins...
  70. ncbi Differential expression of alpha A- and alpha B-crystallin during murine ocular development
    M L Robinson
    Department of Cell Biology, Baylor College of Medicine, Houston, Texas 77030, USA
    Invest Ophthalmol Vis Sci 37:2276-84. 1996
    ..To compare the temporal and spatial expression patterns of alpha A- and alpha B-crystallin mRNA during ocular development...
  71. ncbi Crystallins, genes and cataract
    Suraj P Bhat
    Jules Stein Eye Institute and Brain Research Institute, Geffen School of Medicine at UCLA, Los Angeles, CA 90077 7000, USA
    Prog Drug Res 60:205-62. 2003
    ..b>Crystallins (alpha, beta, and gamma) provide transparency by dint of their high concentration, but it is debatable whether ..
  72. ncbi Evolution of a protein superfamily: relationships between vertebrate lens crystallins and microorganism dormancy proteins
    G Wistow
    National Eye Institute, National Institutes of Health, Bethesda, Maryland 20982
    J Mol Evol 30:140-5. 1990
    ..encystment-specific protein of Physarum polycephalum, is probably structurally related to the beta- and gamma-crystallins, vertebrate ocular lens proteins, and to Protein S, a sporulation-specific protein of Myxococcus xanthus...
  73. ncbi Calcium binding properties of gamma-crystallin: calcium ion binds at the Greek key beta gamma-crystallin fold
    B Rajini
    Centre for Cellular and Molecular Biology, Uppal Road, Hyderabad-500007, India
    J Biol Chem 276:38464-71. 2001
    The beta- and gamma-crystallins are closely related lens proteins that are members of the betagamma-crystallin superfamily, which also include many non-lens members...
  74. ncbi Mammalian forebrain ketimine reductase identified as μ-crystallin; potential regulation by thyroid hormones
    André Hallen
    Department of Chemistry and Biomolecular Sciences, Macquarie University, North Ryde, New South Wales, Australia
    J Neurochem 118:379-87. 2011
    ..This newly demonstrated enzymatic activity identifies a new role for thyroid hormones in regulating mammalian amino acid metabolism, and a possible reciprocal role of enzyme activity regulating bioavailability of intracellular T3...
  75. ncbi Protein composition of the intranuclear inclusions of FXTAS
    C K Iwahashi
    Department of Biochemistry and Molecular Medicine, University of California, Davis, CA, USA
    Brain 129:256-71. 2006
    ..The list of proteins includes at least two RNA binding proteins, heterogeneous nuclear ribonucleoprotein A2 and muscle blind-like protein 1, which are possible mediators of the RNA gain-of-function in FXTAS...
  76. ncbi Beta-crystallins insolubilized by calpain II in vitro contain cleavage sites similar to beta-crystallins insolubilized during cataract
    L L David
    Department of Oral Molecular Biology, Oregon Health Sciences University, Portland 97201
    FEBS Lett 324:265-70. 1993
    ..These data suggested that calpain II caused beta-crystallin insolubilization during cataract formation, and indicated that the process can be mimicked in vitro...
  77. pmc In vivo carbamylation and acetylation of water-soluble human lens alphaB-crystallin lysine 92
    V N Lapko
    Department of Chemistry, University of Nebraska, Lincoln, Nebraska 68588 0304, USA
    Protein Sci 10:1130-6. 2001
    ..Lys 92 was acetylated in one population and carbamylated in another. Although carbamylation of lens crystallins has been predicted, this is the first documentation of in vivo carbamylation of a specific site...
  78. ncbi Small heat shock proteins, the cytoskeleton, and inclusion body formation
    M W Head
    The National CJD Surveillance Unit, Western General Hospital, Edinburgh
    Neuropathol Appl Neurobiol 26:304-12. 2000
    ....
  79. pmc Association properties of betaB1- and betaA3-crystallins: ability to form heterotetramers
    May P Chan
    National Eye Institute and National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
    Biochemistry 47:11062-9. 2008
    As major constituents of the mammalian lens, beta-crystallins associate into dimers, tetramers, and higher-order complexes to maintain lens transparency and refractivity...
  80. ncbi Differential phosphorylation of alpha-A crystallin in human lens of different age
    L J Takemoto
    Division of Biology, Kansas State University, Manhattan 66506, USA
    Exp Eye Res 62:499-504. 1996
    ....
  81. ncbi Zeta-crystallin from guinea pig lens is capable of functioning catalytically as an oxidoreductase
    P V Rao
    Laboratory of Mechanisms of Ocular Diseases, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892
    Arch Biochem Biophys 284:181-5. 1991
    ..This activity was abolished by heat inactivation and immunoadsorption of the protein. Dicumarol, Cibacron blue, manganese, and sulfhydryl reagents were inhibitory...
  82. ncbi Expression of alphaB-crystallin in a mouse model of inherited retinal degeneration
    S E Jones
    British Retinitis Pigmentosa Society Laboratory, Department of Pharmacology, GKT, The Rayne Institute, St Thomas Hospital, London, UK
    Neuroreport 9:4161-5. 1998
    ....
  83. ncbi Alpha B-crystallin in skeletal muscle: purification and localization
    Y Atomi
    Department of Sports Sciences, College of Arts and Sciences, University of Tokyo
    J Biochem 110:812-22. 1991
    ..Muscle alpha B-crystallin is tentatively proposed to be a myofibril-stabilizing protein, based upon its extraction characteristics, localization, and amino acid sequence...
  84. ncbi Aggregation and fibrillation of eye lens crystallins by homocysteinylation; implication in the eye pathological disorders
    Sima Khazaei
    Protein Chemistry Laboratory PCL, Department of Biology, Shiraz University, Shiraz, Iran
    Protein J 31:717-27. 2012
    ..Therefore in this study the effects of homocysteinylation on aggregation and fibrillation of lens crystallins were studied using spectroscopic techniques, SDS-PAGE and western blot analysis...
  85. ncbi Prenatal lens development in connexin43 and connexin50 double knockout mice
    T W White
    Department of Physiology and Biophysics, State University of New York, Stony Brook 11794 8661, USA
    Invest Ophthalmol Vis Sci 42:2916-23. 2001
    ..To determine the roles of intercellular communication in embryonic eye growth and development, mice with a targeted deletion of the Cx43 gene were examined, and mice without both Cx43 and Cx50 were generated and analyzed...
  86. ncbi Proteomic analysis of human lens epithelial cells exposed to microwaves
    Hong Wu Li
    Eye Center, Affiliated Second Hospital, College of Medicine, Zhejiang University, Hangzhou, China
    Jpn J Ophthalmol 51:412-6. 2007
    ..To study proteomic changes in human lens epithelial cells (HLECs) exposed to 1800-MHz Global System for Mobile Communication (GSM)-like microwaves...
  87. ncbi Nerve cells expressing heat-shock proteins in Parkinson's disease
    H Braak
    Department of Clinical Neuroanatomy, J W Goethe University, Frankfurt Main, Germany
    Acta Neuropathol 102:449-54. 2001
    ..Nevertheless, most of the telencephalic alphaB-crystallin-immunopositive neurons refrain from developing Lewy bodies and Lewy neurites and, vice versa, most of the nerve cells containing Lewy bodies do not accumulate alphaB-crystallin...
  88. pmc Aldehyde dehydrogenases: from eye crystallins to metabolic disease and cancer stem cells
    Vasilis Vasiliou
    Department of Pharmaceutical Sciences, University of Colorado Anschutz Medical Campus, Aurora, CO 80445, USA
    Chem Biol Interact 202:2-10. 2013
    ..The latter include functioning as structural elements in the eye (crystallins) and as binding molecules to endobiotics and xenobiotics...
  89. ncbi Crystallins in the eye: Function and pathology
    Usha P Andley
    Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, MO, 63110, USA
    Prog Retin Eye Res 26:78-98. 2007
    b>Crystallins are the predominant structural proteins in the lens that are evolutionarily related to stress proteins...
  90. ncbi Proteomic analysis of the oxidation of cysteine residues in human age-related nuclear cataract lenses
    Peter G Hains
    Save Sight Institute, University of Sydney, Sydney, NSW 2001, Australia
    Biochim Biophys Acta 1784:1959-64. 2008
    ..of age-related nuclear cataract (ARNC), however, little is known about the specific sites of oxidation of the crystallins. We investigated cysteine residues in ARNC lenses and compared them with age-matched normal lenses...
  91. ncbi cDNA and deduced protein sequence for the beta B1-crystallin polypeptide of the chicken lens. Conservation of the PAPA sequence
    J F Hejtmancik
    J Biol Chem 261:982-7. 1986
    ..This appears to be associated with microheterogeneity of the polyadenylation site by comparison of two cDNA clones, suggesting an additional level at which diversity in crystallin gene expression may arise...
  92. ncbi Effect of long-term dietary manipulation on the aggregation of rat lens crystallins: role of alpha-crystallin chaperone function
    G Bhanuprakash Reddy
    National Institute of Nutrition ICMR, Hyderabad, India
    Mol Vis 8:298-305. 2002
    To investigate the effect of food, protein, and vitamin restriction on the susceptibility of lens crystallins to aggregation and chaperone activity of alpha-crystallin.
  93. pmc Tau-crystallin/alpha-enolase: one gene encodes both an enzyme and a lens structural protein
    G J Wistow
    Laboratory of Molecular and Developmental Biology, National Eye Institute, Bethesda, Maryland 20892
    J Cell Biol 107:2729-36. 1988
    ..tau-Crystallin/alpha-enolase is an example of a multifunctional protein playing two very different roles in evolution but encoded by a single gene...
  94. pmc Proteomics analysis of water insoluble-urea soluble crystallins from normal and dexamethasone exposed lens
    Lin Wang
    Eye Hospital, The First Affiliated Hospital, Harbin Medical University, Harbin, China
    Mol Vis 17:3423-36. 2011
    The aim of this study was to identify glucocorticoid induced cataracts (GIC)-specific modified water insoluble-urea soluble (WI-US) crystallins and related changes after rat lens were exposed to dexamethasone (Dex).
  95. ncbi Calcium-binding crystallins from Yersinia pestis. Characterization of two single betagamma-crystallin domains of a putative exported protein
    Maroor K Jobby
    Center for Cellular and Molecular Biology, Uppal Road, Hyderabad 500007, India
    J Biol Chem 280:1209-16. 2005
    ..We suggest that this protein might be involved in calcium-dependent processes, such as stress response or physiology in the Yersinia genus, similar to its microbial relatives and mammalian lens crystallins.
  96. ncbi Lens crystallins of invertebrates--diversity and recruitment from detoxification enzymes and novel proteins
    S I Tomarev
    Laboratory of Molecular and Developmental Biology, National Eye Institute, National Institutes of Health, Bethesda, MD 20892 2730, USA
    Eur J Biochem 235:449-65. 1996
    The major proteins (crystallins) of the transparent, refractive eye lens of vertebrates are a surprisingly diverse group of multifunctional proteins. A number of lens crystallins display taxon-specificity...
  97. ncbi Purification and characterization of a 20-kDa protein that is highly homologous to alpha B crystallin
    K Kato
    Department of Biochemistry, Institute for Developmental Research, Aichi Prefectural Colony, Japan
    J Biol Chem 269:15302-9. 1994
    ..These results suggest that p20 is related to stress proteins...
  98. ncbi Heat-induced conformational change of human lens recombinant alphaA- and alphaB-crystallins
    J J Liang
    Center for Ophthalmic Research, Brigham and Women s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Mol Vis 6:10-4. 2000
    ..To determine which component of lens alpha-crystallin is responsible for heat-induced transition, conformational change and high molecular weight (HMW) aggregation...
  99. ncbi Phosphorylation of alpha-crystallin in rat lenses is stimulated by H2O2 but phosphorylation has no effect on chaperone activity
    K Wang
    Department of Ophthalmology, College of Physicians and Surgeons of Columbia University, New York, NY 10032, USA
    Exp Eye Res 61:115-24. 1995
    ..alpha A and alpha B chains as measured by suppressing the thermally induced aggregation of beta low or gamma crystallins. It was also found that the alpha A aggregates are more effective chaperones than the alpha B aggregates...
  100. ncbi Study of the interaction between triplet riboflavin and the alpha-, betaH- and betaL-crystallins of the eye lens
    Gabriela Viteri
    , , Santiago, Chile
    Photochem Photobiol 77:535-40. 2003
    ..photolysis studies of riboflavin (RF) were carried out in the presence and absence of alpha-, betaH- and betaL-crystallins of bovine eye lens...
  101. pmc Shotgun proteomic analysis of S-thiolation sites of guinea pig lens nuclear crystallins following oxidative stress in vivo
    Frank J Giblin
    Eye Research Institute, Oakland University, Rochester, MI 48309, USA
    Mol Vis 19:267-80. 2013
    To compare levels of S-glutathiolation and S-cysteinylation occurring at more than 60 cysteine residues of 12 different guinea pig lens water-soluble nuclear crystallins following treatment of the animals with hyperbaric oxygen (HBO).

Research Grants65

  1. Alpha B crystallin as a novel therapeutic for SIRS and Sepsis
    Jonathan B Rothbard; Fiscal Year: 2011
    ....
  2. Therapeutic Effect of a Novel Antioxidant on Degenerative Eye Disorders
    Nuran Ercal; Fiscal Year: 2012
    ..We will also study protein oxidation of crystallins, lipid peroxidation, and protein bound GSH and cysteine...
  3. Prevention of Retinal Degeneration by Crystallins in Experimental Uveitis
    Narsing A Rao; Fiscal Year: 2011
    ..Moreover, it indicates that unlike other tissues, the photoreceptors utilize 1A crystallins selectively in the prevention of oxidative stress-mediated damage in early EAU, a novel protective mechanism ..
  4. DEVELOPMENT AND MAINTENANCE OF LENS TRANSPARENCY
    JOHN IRWIN CLARK; Fiscal Year: 2013
    ..The lens is used for these studies because of its unique accessibility for the in vivo investigation of protein unfolding and aggregation diseases in aging and the well known protective effects of ?B crystallin. ..
  5. 5500 Q-Trap Mass Spectrometer
    Stephen Barnes; Fiscal Year: 2010
    ..in activated neutrophils, the enzymes in glycolysis and energy generating mitochondrial pathways, and the lens crystallins in aging...
  6. Development of Small Heat Shock Proteins as Therapeutic Agents in the Eye
    JONATHAN MARK PETRASH; Fiscal Year: 2010
    ..Our proposed studies could substantially improve the quality of life of affected patients by preventing vision loss and associated financial burdens due to lost productivity and increased medical expenses. ..
  7. Gene Regulation by and of LEDGF
    Dhirendra P Singh; Fiscal Year: 2012
    ....
  8. AMINO-CARBONYL REACTIONS IN THE AGING HUMAN LENS
    Vincent M Monnier; Fiscal Year: 2013
    ..Aim 1 will test the hypothesis that 1) carbonyl attack onto arginine residues of human and mouse gamma-crystallins results in altered protein charge, deguanylation into ornithine at identical sites, protein unfolding, exposure ..
  9. Aging specific gene expression in Drosophila
    JOHN GERARD TOWER; Fiscal Year: 2013
    ..By studying gene expression in aging Drosophila, we hope to better understand the basic mechanisms of aging, and to ultimately develop interventions for human aging-related diseases. ..
  10. Progressive impact of diabetes on retinal neuroprotection by ?-crystallins
    Patrice E Fort; Fiscal Year: 2013
    DESCRIPTION (provided by applicant):?A- and ?B-crystallins are highly upregulated in the retina during diabetes;however, there is a fundamental gap in our knowledge regarding the role of this sustained increase on retinal cell death and ..
  11. Regulation of Eye Growth and Development by the Lens
    William R Jeffery; Fiscal Year: 2012
    ..Using the blind cavefish as a model, this study is designed to provide new information about the role of the ocular lens in coordinating eye development. The results will provide insights into abnormal eye development. ..
  12. The functions and mechanism of SHSP degradation in Escherichia coli
    ELLEN FRANCES VIEUX; Fiscal Year: 2011
    ....
  13. Epigenetic regulation of lens fiber cell differentiation: The role of DNA methyla
    Michael L Robinson; Fiscal Year: 2013
    ..Conversely many other genes including Aquaporin0, 2- and 3-crystallins and lens-specific beaded filament proteins CP49 and filensin are not expressed in lens epithelial cells but are ..
  14. Molecular characterization of congenital cataract
    Elena V Semina; Fiscal Year: 2013
    ..Name Organization Role on Project Elena V. Semina, PhD Medical College of Wisconsin PI Disclosure Permission Statement. Applicable to SBIR/STTR Only. See instructions. Yes No PHS 398 (Rev. 05/01) Page _2___ Form Page 2 ..
  15. Noninvasive Visualization and Analysis of Organ Systems using OCT and Digital Fun
    Steven J Pittler; Fiscal Year: 2010
    ..The use of this new technology will dramatically advance our ability to correlate structure and function in living animals and in isolated tissues and will ultimately lead to new ways to treat diseases in humans. ..
  16. Assessment of Corneal Fibroblast Biomechanical Behavior
    W Matthew Petroll; Fiscal Year: 2013
    ..factors and Rho kinase activation regulate expression of normal and fibrotic keratocyte markers and corneal crystallins in 3-D culture;factors that are directly associated with maintenance or loss of corneal transparency during in ..
  17. ROLE OF TOXINS IN ENDOPHTHALMITIS
    MEREDITH GREGORY-KSANDER; Fiscal Year: 2012
    ..It may also have the extrinsic value of leading to new strategies for the treatment and prevention of antibiotic resistant infection at other sites as well. ..
  18. Regulation of cell homeostasis by fbx4
    JOHN ALAN DIEHL; Fiscal Year: 2013
    ....
  19. PROTEINS OF NORMAL AND CATARACTOUS LENSES
    FRANK JOSEPH GIBLIN; Fiscal Year: 2012
    ..Aim 1 will investigate the mechanism of O2-induced disulfide-crosslinking of lens crystallins, a modification strongly associated with human nuclear cataract...
  20. Transcriptional control of the mouse aA-crystallin locus
    Ales Cvekl; Fiscal Year: 2013
    ....
  21. Induced Autoantigen Expression Exacerbates EAE
    Kenneth L Bost; Fiscal Year: 2011
    ..Specifically, we will question whether an autoimmune response against crystallin alpha-beta following an infection might contribute to the exacerbation of clinical disease in this animal model of multiple sclerosis. ..
  22. Mechanisms of lens growth regulation
    Xiaohua Gong; Fiscal Year: 2013
    ..Anticipated results may be useful for developing a new strategy to inhibit lens size increase, thus delaying or preventing presbyopia and age-related cataract. ..
  23. Mechanistic basis and potential therapies for myosin storage myopathy
    Sanford I Bernstein; Fiscal Year: 2013
    ..This multifaceted approach will provide novel insights into the developmental and biophysical bases of MSM and yield potential therapeutic approaches that may be useful for treating MSM and other inclusion body diseases. ..
  24. Pax6 as a key regulator of lens development
    Ales Cvekl; Fiscal Year: 2013
    ..abstract_text> ..
  25. Molecular mechanism of senile cardiac amyloidosis
    Lawreen H Connors; Fiscal Year: 2013
    ....
  26. Reductive Stress in the Pathogenesis of R120GCryAB Cardiomyopathy
    Ivor J Benjamin; Fiscal Year: 2010
    ..Our discovery establishes that G6PD plays a key role and supports the rationale for further studies that might lead to new therapies for human cardiac and degenerative diseases. ..
  27. A novel bA3/A1-crystallin gene mutation results in persistent fetal vasculature
    Debasish Sinha; Fiscal Year: 2013
    ....
  28. Generating molecular markers that selectively label urothelial sub-populations
    CATHY LEE MENDELSOHN; Fiscal Year: 2013
    ..The relationships that we have developed with GUDMAP collaborators and experience with expression analysis in the LUT will be an advantage as we undertake the proposed studies. ..
  29. Structural studies of substrate recognition and specificity by the SCF ubiquitin
    Bing Hao; Fiscal Year: 2013
    ....
  30. AD LENS PATHOLOGY: BIOCHEMISTRY & DIAGNOSTIC IMAGING
    Lee E Goldstein; Fiscal Year: 2010
    ..We will investigate AB binding to cytosolic structural proteins (a-, b-, and y-crystallins) and study lens protein aggregation in the presence of AB species, metal chelators, and gases (air, 02, Ar)...
  31. Novel Role of Corneal Crystallins as Modulators of Cell Growth and Transparency
    Vasilis Vasiliou; Fiscal Year: 2011
    ..and also in a taxon-specific pattern, several cytosolic enzyme/proteins that have been named 'corneal crystallins', based on the similarity with the lens crystallins...
  32. Mechanisms of Glucocorticoid Action in Podocytes
    RICHARD FREDERICK RANSOM; Fiscal Year: 2011
    ..Relevance: Identification of a podocyte mechanism of action of glucocorticoids in NS would permit the development of more targeted and less toxic therapies. ..
  33. The Regulation of Lens Fiber Cell Differentiation
    Melinda K Duncan; Fiscal Year: 2012
    ..in gene activation, these studies will allow us to understand how the enormous transcriptional activity of crystallins is accomplished...
  34. Structure/Function Studies of Small Heat Shock Proteins
    Rachel E Klevit; Fiscal Year: 2013
    ..The proposed studies build on the burgeoning progress towards understanding the structural biology of ?B and the important family of sHSPs afforded by developments of techniques capable of studying large heterogenous species. ..
  35. Compounds blocking crystallin aggregation in vitro;path to anti-cataract agents
    JONATHAN ALAN KING; Fiscal Year: 2013
    ..In the last project period, in vitro systems were developed in which partially unfolded human crystallins aggregated into light-scattering complexes at pH7 and amyloid fibers at low pH...
  36. Molecular Analysis of Microdissected Human Lenses
    Marc Kantorow; Fiscal Year: 2013
    ..The information gained from this work will have a major impact on the rational design of therapeutics that could prevent cataract formation. ..
  37. Crystallin-Derived Mini-Chaperones as Protein Aggregation Inhibitors
    Krishna K Sharma; Fiscal Year: 2011
    ..The goals of this research are to develop and test lens protein 1-crystallin-derived peptides as active mini-chaperones capable of suppressing protein aggregation. ..
  38. Regulation of Apoptotic Signaling Pathways by Alpha-Crystallins in the Ocular Len
    David W Li; Fiscal Year: 2010
    Recent studies have shown that a-crystallins are strong anti-apoptotic regulators, preventing apoptosis induced by a large number of stress factors...
  39. Alpha-Crystallin Function in Lens Biology
    Usha P Andley; Fiscal Year: 2012
    ..Lens crystallins account for 90% of the total lens proteins and play a key role in lens transparency...
  40. Crystallin-Derived Anti-Chaperones in the Lens
    Krishna K Sharma; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Changes in the structure of crystallins, the major lens protein, are thought to account for the loss of lens transparency that occurs with aging...
  41. Proteinase Inhibitors and Crystallin Fragments in Cataract
    OM PRAKASH SRIVASTAVA; Fiscal Year: 2013
    ..Deamidation and truncations of crystallins are identified as the most abundant post-translational modifications (PTMs), and are believed to be the major ..
  42. Metastable Crystallins: Structure and Stabilization
    Krishna K Sharma; Fiscal Year: 2013
    ..During aging, lens crystallins undergo truncation and these modifications correlate with lens crystallin aggregations responsible for light ..
  43. AN EXPERIMENTAL APPROACH TO MACULOPATHY
    David R Hinton; Fiscal Year: 2013
    ..abstract_text> ..
  44. Molecular Basis for Lens Transparency
    Hassane S McHaourab; Fiscal Year: 2013
    ....
  45. Membrane Protein Co- Crystallization with Highly Crystalline and Soluble Proteins
    Gregory A Weiss; Fiscal Year: 2013
    ..In summary, this proposal will define new approaches to protein engineering and molecular recognition, through development of new fusion proteins and their use in the recognition of membrane proteins. ..
  46. Conformation and Dynamics of Cataract Mutants of human gammaD crystallin
    Angela M Gronenborn; Fiscal Year: 2013
    ..b>Crystallins are ideally suited for detailed studies of protein aggregation: they are small;numerous X-ray structures are ..
  47. POST TRANSCRIPTIONAL CONTROL OF GENE EXPRESSION IN THE LENS (LENS GENE EXPRESSION
    SALIL LACHKE; Fiscal Year: 2013
    ..These include cytoplasmic crystallins, specialized membrane proteins and intermediate filaments...
  48. Development of a diagnostic for latent TB
    Frederick D Quinn; Fiscal Year: 2012
    ..Such a test has been identified as a high priority goal to facilitate a National plan to eliminate tuberculosis in the United States. ..
  49. Solid-state NMR methods for investigating native and aggregated eye lens proteins
    RACHEL WAGNER MARTIN; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): The crystallins, which maintain the transparency of the eye lens by providing a well-defined gradient of refractive index, are a fascinating and medically relevant group of proteins...
  50. Phase boundaries and liquid structure of concentrated eye lens protein mixtures
    George Thurston; Fiscal Year: 2013
    ..detailed experimental and theoretical investigation of orientation- dependent interactions between gamma crystallins, while focusing in part on screened, charge-regulated electrostatic interactions, and (3) systematically ..
  51. Gamma crystallin modifications &mechanisms of lens opacity
    Jayanti Pande; Fiscal Year: 2013
    ..objective of this proposal is to determine the molecular mechanisms by which modifications of the [unreadable]-crystallins (genetic or age-onset), lead to cataract formation...
  52. MODIFICATION OF ALPHA-CRYSTALLIN CHAPERONE FUNCTION
    Edathara C Abraham; Fiscal Year: 2013
    ..More than half of the muitants occur in crystallins, in [unreadable]A- and [unreadable]B-crystallins mostly...
  53. ANALYSIS OF THE ENZYMATIC PROPERTIES OF CRYSTALLIN
    WILLIAM O BRIEN; Fiscal Year: 1990
    ..Such proteins can be synthesized by splicing pieces of the cDNA molecules together at common restriction sites. This approach will be complemented with site directed mutagenesis studies to define the role of individual amino acids...
  54. A CRYSTALLIN MUTATION WITH ABNORMAL ASTROCYTES AND RETINAL VESSELS
    Debasish Sinha; Fiscal Year: 2010
    ..We believe that studies on the Nuc1 rat will provide new insights into the cellular and molecular mechanisms that regulate vascular development including the molecular interactions among neurons, astrocytes and endothelial cells. ..
  55. MECHANISM OF SELENIUM INDUCED CATARACT
    Thomas Shearer; Fiscal Year: 2002
    ..protease (m-calpain), loss of the N-terminal extensions on beta-crystallin polypeptides, insolubilization of crystallins, and opacity...
  56. Notch Signaling and Lens Development
    Pumin Zhang; Fiscal Year: 2010
    ..We will validate this initial finding with in vitro and in vivo experiments. ..
  57. LENS PROTEIN GLYCATION AND CATARACT DEVELOPMENT
    EDATHARA ABRAHAM; Fiscal Year: 1999
    b>Crystallins of the eye lens are long-lived proteins because they hardly turn over. Such proteins are constantly subjected to posttranslational modifications such as nonenzymatic glycosylation (glycation)...
  58. Alpha Crystallin Function in the Zebrafish
    Mason Posner; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): The vertebrate ??crystallins play an essential role in lens development and the maintenance of lens transparency...
  59. TARGETED ABLATION IN EYE AND BRAIN
    S Potter; Fiscal Year: 1993
    ..The studies will also provide murine model systems for specific developmental defects such s retinitis pigmentosa...
  60. Alpha-Crystallin and Cataractogensis
    Joseph Horwitz; Fiscal Year: 2009
    ..These are excellent models for understanding the biochemical and biophysical changes that occur in age-related human cataract. ..
  61. The role of alphaB-crystallin in chemoresistance of retinoblastoma
    MERIDETH KREVOSKY; Fiscal Year: 2007
    ..Our long-term goal is to prevent loss of vision and to improve the quality of life for retinoblastoma patients. [unreadable] [unreadable] [unreadable]..
  62. LENS PROTEIN ALPHA-CRYSTALLIN B CHAIN
    James Goldman; Fiscal Year: 1999
    ..2. further define how aB-crystallinprotects cells from hypertonic and other stresses, 3. explore mechanisms of Rosenthal Fiber production. ..
  63. Role of Deamidation in Human Beta-Crystallin Structure
    KIRSTEN JEANNE LAMPI; Fiscal Year: 2012
    ..the last grant cycle, we convincingly demonstrated that deamidation led to soluble aggregates, destabilized b-crystallins, altered interactions between b-subunits, and increased the a-crystallin needed to chaperone b-crystallins...
  64. Beta-B1 Crystallin - a New Candidate Uveitis Autoantigen
    Lynn Gordon; Fiscal Year: 2003
    ..abstract_text> ..
  65. PROTEIN GLYCATION--STRUCTURE AND STABILITY OF PRODUCTS
    Ajay Pande; Fiscal Year: 1993
    ..Among the lens crystallins, the gamma-crystallins are maximally glycated, both in vivo and in vitro...