Genomes and Genes
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.
Publications304 found, 100 shown here
- Mutation analysis of CRYAA, CRYGC, and CRYGD associated with autosomal dominant congenital cataract in Brazilian familiesAlessandro 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...
- Comprehensive mutational screening in a cohort of Danish families with hereditary congenital cataractLars 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...
- Congenital cataracts and their molecular geneticsJ 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...
- Lens aging: effects of crystallinsK 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 ..
- 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...
- The retinal proteome in experimental diabetic retinopathy: up-regulation of crystallins and reversal by systemic and periocular insulinPatrice 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...
- Regulation of gene expression by Pax6 in ocular cells: a case of tissue-preferred expression of crystallins in lensAles 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...
- Cytoskeletal competence requires protein chaperonesRoy Quinlan
Department of Biological Sciences, South Road, Durham DH1 3LE, UK
Prog Mol Subcell Biol 28:219-33. 2002
- Genomic analyses reveal global functional alterations that promote tumor growth and novel tumor suppressor genes in natural killer-cell malignanciesJ 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...
- A proteome map of the zebrafish (Danio rerio) lens reveals similarities between zebrafish and mammalian crystallin expressionMason 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...
- Identification of CRYM as a candidate responsible for nonsyndromic deafness, through cDNA microarray analysis of human cochlear and vestibular tissuesSatoko 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...
- sHSP in the eye lens: crystallin mutations, cataract and proteostasisAlice 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...
- Eye evolution: lens and cornea as an upgrade of animal visual systemKristyna 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.
- Mutation analysis of 12 genes in Chinese families with congenital cataractsWenmin 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...
- Simultaneous racemization and isomerization at specific aspartic acid residues in alpha B-crystallin from the aged human lensN 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...
- Decoding the molecular design principles underlying Ca(2+) binding to βγ-crystallin motifsAmita 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...
- Characterization of the G91del CRYBA1/3-crystallin protein: a cause of human inherited cataractM 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 ..
- A deletion mutation in the betaA1/A3 crystallin gene ( CRYBA1/A3) is associated with autosomal dominant congenital nuclear cataract in a Chinese familyYanhua 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...
- Transgene overexpression of alphaB crystallin confers simultaneous protection against cardiomyocyte apoptosis and necrosis during myocardial ischemia and reperfusionP 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...
- The ageing lens and cataract: a model of normal and pathological ageingR 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...
- Ageing and vision: structure, stability and function of lens crystallinsHans Bloemendal
Department of Biochemistry, University of Nijmegen, 6500HB, The Netherlands
Prog Biophys Mol Biol 86:407-85. 2004The 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...
- Crystallins of the beta/gamma-superfamily mimic the effects of lens injury and promote axon regenerationDietmar 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...
- Muscle develops a specific form of small heat shock protein complex composed of MKBP/HSPB2 and HSPB3 during myogenic differentiationY 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...
- AIM1, a novel non-lens member of the betagamma-crystallin superfamily, is associated with the control of tumorigenicity in human malignant melanomaM 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...
- Molecular characteristics of inherited congenital cataractsBingyu 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 ..
- A novel mutation in AlphaA-crystallin (CRYAA) caused autosomal dominant congenital cataract in a large Chinese familyFeng 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...
- Mutation G61C in the CRYGD gene causing autosomal dominant congenital coralliform cataractsFeifeng 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...
- Cloning, expression, and chaperone-like activity of human alphaA-crystallinU 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....
- Vitreoretinal influences on lens function and cataractDavid 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...
- Lens proteomics: analysis of rat crystallins when lenses are exposed to dexamethasoneLin Wang
Harbin Medical University, Harbin, China
Mol Biosyst 8:888-901. 2012To identify glucocorticoid induced cataract (GIC)-specific modified crystallins and related changes, we analyzed rat crystallins and related changes in lenses exposed to dexamethasone (Dex)...
- βA3/A1-crystallin is required for proper astrocyte template formation and vascular remodeling in the retinaDebasish 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....
- Lens gene expression analysis reveals downregulation of the anti-apoptotic chaperone alphaA-crystallin during cavefish eye degenerationAllen 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, ..
- Differential regulation of gamma-crystallin genes during mouse lens developmentM 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.
- Identification and characterization of the enzymatic activity of zeta-crystallin from guinea pig lens. A novel NADPH:quinone oxidoreductaseP 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 ..
- Bmp signaling is required for development of primary lens fiber cellsSonya 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...
- Phosphorylation-induced change of the oligomerization state of alpha B-crystallinH 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...
- X-ray analysis of the eye lens protein gamma-II crystallin at 1.9 A resolutionG 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 ..
- Colloidal characterization and thermodynamic stability of binary eye lens protein mixturesN 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...
- Sulfur in human crystallinsDurga Srikanthan
Department of Crystallography, Birkbeck College, University of London, Malet Street, London WC1E 7HX, UK
Exp Eye Res 79:823-31. 2004Molecular 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...
- Up-regulation of crystallins is involved in the neuroprotective effect of wolfberry on survival of retinal ganglion cells in rat ocular hypertension modelKin 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...
- Conversion of arginine into ornithine by advanced glycation in senescent human collagen and lens crystallinsDavid 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...
- A comprehensive analysis of the expression of crystallins in mouse retinaJinghua Xi
Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, Missouri 63110, USA
Mol Vis 9:410-9. 2003b>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...
- Connexin 46 and connexin 50 in selenite cataractC R Fleschner
A T Still University of Health Sciences, Kirksville College of Osteopathic Medicine, MO 63501 1443, USA
Ophthalmic Res 38:24-8. 2006....
- Tight binding of proteins to membranes from older human cellsRoger 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 ..
- Intense light exposure changes the crystallin content in retinaHirokazu 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.
- Pax6 is essential for lens fiber cell differentiationOhad 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...
- Energetics of domain-domain interactions and entropy driven association of beta-crystallinsY V Sergeev
National Eye Institute, National Institutes of Health, Bethesda, Maryland 20982, USA
Biochemistry 43:415-24. 2004Beta-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...
- Unfolding crystallins: the destabilizing role of a beta-hairpin cysteine in betaB2-crystallin by simulation and experimentJames T MacDonald
School of Crystallography, Birkbeck College, Malet Street, London, WC1E 7HX, UK
Protein Sci 14:1282-92. 2005The thermodynamic and kinetic stabilities of the eye lens family of betagamma-crystallins are important factors in the etiology of senile cataract...
- 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...
- 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 mannerA 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...
- Glycation by ascorbic acid oxidation products leads to the aggregation of lens proteinsMikhail 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 ..
- Characterization of covalent multimers of crystallins in aging human lensesOm 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...
- Vitamin C mediates chemical aging of lens crystallins by the Maillard reaction in a humanized mouse modelXingjun 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 ..
- Mechanisms involved in the protection of UV-induced protein inactivation by the corneal crystallin ALDH3A1Tia 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...
- A novel non-lens betagamma-crystallin and trefoil factor complex from amphibian skin and its functional implicationsShu 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. 2008In vertebrates, non-lens betagamma-crystallins are widely expressed in various tissues, but their functions are unknown...
- Mutation in the βA3/A1-crystallin gene impairs phagosome degradation in the retinal pigmented epithelium of the ratJ 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...
- Genetics of crystallins: cataract and beyondJochen Graw
Helmholtz Center Munich, German Research Center for Environmental Health, Institute of Developmental Genetics, D 85764 Neuherberg, Germany
Exp Eye Res 88:173-89. 2009The crystallins were discovered more than 100 years ago by Mörner (1893. Untersuchungen der Proteinsubstanzen in den lichtbrechenden Medien des Auges. Z. Physiol. Chem...
- Review: A case for corneal crystallinsJ 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. 2000It 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...
- Alpha-crystallin-type heat shock proteins: socializing minichaperones in the context of a multichaperone networkFranz Narberhaus
Institut fur Mikrobiologie, Eidgenossische Technische Hochschule, CH 8092 Zurich, Switzerland
Microbiol Mol Biol Rev 66:64-93; table of contents. 2002Alpha-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...
- Formation of amyloid fibrils in vitro by human gammaD-crystallin and its isolated domainsKaterina 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 ..
- Genealogy of the alpha-crystallin--small heat-shock protein superfamilyW 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...
- Dual roles for Pax-6: a transcriptional repressor of lens fiber cell-specific beta-crystallin genesM K Duncan
Department of Biological Sciences, The University of Delaware, Newark, Delaware 19716, USA
Mol Cell Biol 18:5579-86. 1998....
- Cleavage of the C-terminal serine of human alphaA-crystallin produces alphaA1-172 with increased chaperone activity and oligomeric sizeAtya 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...
- CRYGD gene analysis in a family with autosomal dominant congenital cataract: evidence for molecular homogeneity and intrafamilial clinical heterogeneity in aculeiform cataractJuan 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...
- A novel deletion variant of gammaD-crystallin responsible for congenital nuclear cataractLi 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...
- AlphaB-crystallin in lens development and muscle integrity: a gene knockout approachJ 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...
- R120G alphaB-crystallin promotes the unfolding of reduced alpha-lactalbumin and is inherently unstableTeresa 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...
- Redox regulation in the lensMarjorie 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...
- Age-dependent deamidation of lifelong proteins in the human lensPeter G Hains
Save Sight Institute, University of Sydney, Sydney, NSW, Australia
Invest Ophthalmol Vis Sci 51:3107-14. 2010Deamidation is a common posttranslational modification in human lens crystallins and may be a key factor in the age-related denaturation of such lifelong proteins...
- Differential expression of alpha A- and alpha B-crystallin during murine ocular developmentM 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...
- Crystallins, genes and cataractSuraj 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 ..
- Evolution of a protein superfamily: relationships between vertebrate lens crystallins and microorganism dormancy proteinsG 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...
- Calcium binding properties of gamma-crystallin: calcium ion binds at the Greek key beta gamma-crystallin foldB Rajini
Centre for Cellular and Molecular Biology, Uppal Road, Hyderabad-500007, India
J Biol Chem 276:38464-71. 2001The beta- and gamma-crystallins are closely related lens proteins that are members of the betagamma-crystallin superfamily, which also include many non-lens members...
- Mammalian forebrain ketimine reductase identified as μ-crystallin; potential regulation by thyroid hormonesAndré 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...
- Protein composition of the intranuclear inclusions of FXTASC 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...
- Beta-crystallins insolubilized by calpain II in vitro contain cleavage sites similar to beta-crystallins insolubilized during cataractL 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...
- In vivo carbamylation and acetylation of water-soluble human lens alphaB-crystallin lysine 92V 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...
- Small heat shock proteins, the cytoskeleton, and inclusion body formationM W Head
The National CJD Surveillance Unit, Western General Hospital, Edinburgh
Neuropathol Appl Neurobiol 26:304-12. 2000....
- Association properties of betaB1- and betaA3-crystallins: ability to form heterotetramersMay 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. 2008As major constituents of the mammalian lens, beta-crystallins associate into dimers, tetramers, and higher-order complexes to maintain lens transparency and refractivity...
- Differential phosphorylation of alpha-A crystallin in human lens of different ageL J Takemoto
Division of Biology, Kansas State University, Manhattan 66506, USA
Exp Eye Res 62:499-504. 1996....
- Zeta-crystallin from guinea pig lens is capable of functioning catalytically as an oxidoreductaseP 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...
- Expression of alphaB-crystallin in a mouse model of inherited retinal degenerationS E Jones
British Retinitis Pigmentosa Society Laboratory, Department of Pharmacology, GKT, The Rayne Institute, St Thomas Hospital, London, UK
Neuroreport 9:4161-5. 1998....
- Alpha B-crystallin in skeletal muscle: purification and localizationY 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...
- Aggregation and fibrillation of eye lens crystallins by homocysteinylation; implication in the eye pathological disordersSima 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...
- Prenatal lens development in connexin43 and connexin50 double knockout miceT 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...
- Proteomic analysis of human lens epithelial cells exposed to microwavesHong 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...
- Nerve cells expressing heat-shock proteins in Parkinson's diseaseH 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...
- Aldehyde dehydrogenases: from eye crystallins to metabolic disease and cancer stem cellsVasilis 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...
- Crystallins in the eye: Function and pathologyUsha 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. 2007b>Crystallins are the predominant structural proteins in the lens that are evolutionarily related to stress proteins...
- Proteomic analysis of the oxidation of cysteine residues in human age-related nuclear cataract lensesPeter 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...
- cDNA and deduced protein sequence for the beta B1-crystallin polypeptide of the chicken lens. Conservation of the PAPA sequenceJ 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...
- Effect of long-term dietary manipulation on the aggregation of rat lens crystallins: role of alpha-crystallin chaperone functionG Bhanuprakash Reddy
National Institute of Nutrition ICMR, Hyderabad, India
Mol Vis 8:298-305. 2002To investigate the effect of food, protein, and vitamin restriction on the susceptibility of lens crystallins to aggregation and chaperone activity of alpha-crystallin.
- Tau-crystallin/alpha-enolase: one gene encodes both an enzyme and a lens structural proteinG 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...
- Proteomics analysis of water insoluble-urea soluble crystallins from normal and dexamethasone exposed lensLin Wang
Eye Hospital, The First Affiliated Hospital, Harbin Medical University, Harbin, China
Mol Vis 17:3423-36. 2011The 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).
- Calcium-binding crystallins from Yersinia pestis. Characterization of two single betagamma-crystallin domains of a putative exported proteinMaroor 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.
- Lens crystallins of invertebrates--diversity and recruitment from detoxification enzymes and novel proteinsS 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. 1996The 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...
- Purification and characterization of a 20-kDa protein that is highly homologous to alpha B crystallinK 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...
- Heat-induced conformational change of human lens recombinant alphaA- and alphaB-crystallinsJ 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...
- Phosphorylation of alpha-crystallin in rat lenses is stimulated by H2O2 but phosphorylation has no effect on chaperone activityK 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...
- Study of the interaction between triplet riboflavin and the alpha-, betaH- and betaL-crystallins of the eye lensGabriela 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...
- Shotgun proteomic analysis of S-thiolation sites of guinea pig lens nuclear crystallins following oxidative stress in vivoFrank J Giblin
Eye Research Institute, Oakland University, Rochester, MI 48309, USA
Mol Vis 19:267-80. 2013To 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).
- Alpha B crystallin as a novel therapeutic for SIRS and SepsisJonathan B Rothbard; Fiscal Year: 2011....
- Therapeutic Effect of a Novel Antioxidant on Degenerative Eye DisordersNuran Ercal; Fiscal Year: 2012..We will also study protein oxidation of crystallins, lipid peroxidation, and protein bound GSH and cysteine...
- Prevention of Retinal Degeneration by Crystallins in Experimental UveitisNarsing 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 ..
- DEVELOPMENT AND MAINTENANCE OF LENS TRANSPARENCYJOHN 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. ..
- 5500 Q-Trap Mass SpectrometerStephen Barnes; Fiscal Year: 2010..in activated neutrophils, the enzymes in glycolysis and energy generating mitochondrial pathways, and the lens crystallins in aging...
- Development of Small Heat Shock Proteins as Therapeutic Agents in the EyeJONATHAN 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. ..
- Gene Regulation by and of LEDGFDhirendra P Singh; Fiscal Year: 2012....
- AMINO-CARBONYL REACTIONS IN THE AGING HUMAN LENSVincent 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 ..
- Aging specific gene expression in DrosophilaJOHN 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. ..
- Progressive impact of diabetes on retinal neuroprotection by ?-crystallinsPatrice E Fort; Fiscal Year: 2013DESCRIPTION (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 ..
- Regulation of Eye Growth and Development by the LensWilliam 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. ..
- The functions and mechanism of SHSP degradation in Escherichia coliELLEN FRANCES VIEUX; Fiscal Year: 2011....
- Epigenetic regulation of lens fiber cell differentiation: The role of DNA methylaMichael 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 ..
- Molecular characterization of congenital cataractElena 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 ..
- Noninvasive Visualization and Analysis of Organ Systems using OCT and Digital FunSteven 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. ..
- Assessment of Corneal Fibroblast Biomechanical BehaviorW 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 ..
- ROLE OF TOXINS IN ENDOPHTHALMITISMEREDITH 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. ..
- Regulation of cell homeostasis by fbx4JOHN ALAN DIEHL; Fiscal Year: 2013....
- PROTEINS OF NORMAL AND CATARACTOUS LENSESFRANK 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...
- Transcriptional control of the mouse aA-crystallin locusAles Cvekl; Fiscal Year: 2013....
- Induced Autoantigen Expression Exacerbates EAEKenneth 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. ..
- Mechanisms of lens growth regulationXiaohua 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. ..
- Mechanistic basis and potential therapies for myosin storage myopathySanford 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. ..
- Pax6 as a key regulator of lens developmentAles Cvekl; Fiscal Year: 2013..abstract_text> ..
- Molecular mechanism of senile cardiac amyloidosisLawreen H Connors; Fiscal Year: 2013....
- Reductive Stress in the Pathogenesis of R120GCryAB CardiomyopathyIvor 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. ..
- A novel bA3/A1-crystallin gene mutation results in persistent fetal vasculatureDebasish Sinha; Fiscal Year: 2013....
- Generating molecular markers that selectively label urothelial sub-populationsCATHY 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. ..
- Structural studies of substrate recognition and specificity by the SCF ubiquitinBing Hao; Fiscal Year: 2013....
- AD LENS PATHOLOGY: BIOCHEMISTRY & DIAGNOSTIC IMAGINGLee 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)...
- Novel Role of Corneal Crystallins as Modulators of Cell Growth and TransparencyVasilis 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...
- Mechanisms of Glucocorticoid Action in PodocytesRICHARD 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. ..
- The Regulation of Lens Fiber Cell DifferentiationMelinda K Duncan; Fiscal Year: 2012..in gene activation, these studies will allow us to understand how the enormous transcriptional activity of crystallins is accomplished...
- Structure/Function Studies of Small Heat Shock ProteinsRachel 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. ..
- Compounds blocking crystallin aggregation in vitro;path to anti-cataract agentsJONATHAN 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...
- Molecular Analysis of Microdissected Human LensesMarc 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. ..
- Crystallin-Derived Mini-Chaperones as Protein Aggregation InhibitorsKrishna 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. ..
- Regulation of Apoptotic Signaling Pathways by Alpha-Crystallins in the Ocular LenDavid W Li; Fiscal Year: 2010Recent studies have shown that a-crystallins are strong anti-apoptotic regulators, preventing apoptosis induced by a large number of stress factors...
- Alpha-Crystallin Function in Lens BiologyUsha P Andley; Fiscal Year: 2012..Lens crystallins account for 90% of the total lens proteins and play a key role in lens transparency...
- Crystallin-Derived Anti-Chaperones in the LensKrishna K Sharma; Fiscal Year: 2013DESCRIPTION (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...
- Proteinase Inhibitors and Crystallin Fragments in CataractOM 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 ..
- Metastable Crystallins: Structure and StabilizationKrishna K Sharma; Fiscal Year: 2013..During aging, lens crystallins undergo truncation and these modifications correlate with lens crystallin aggregations responsible for light ..
- AN EXPERIMENTAL APPROACH TO MACULOPATHYDavid R Hinton; Fiscal Year: 2013..abstract_text> ..
- Molecular Basis for Lens TransparencyHassane S McHaourab; Fiscal Year: 2013....
- Membrane Protein Co- Crystallization with Highly Crystalline and Soluble ProteinsGregory 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. ..
- Conformation and Dynamics of Cataract Mutants of human gammaD crystallinAngela M Gronenborn; Fiscal Year: 2013..b>Crystallins are ideally suited for detailed studies of protein aggregation: they are small;numerous X-ray structures are ..
- POST TRANSCRIPTIONAL CONTROL OF GENE EXPRESSION IN THE LENS (LENS GENE EXPRESSIONSALIL LACHKE; Fiscal Year: 2013..These include cytoplasmic crystallins, specialized membrane proteins and intermediate filaments...
- Development of a diagnostic for latent TBFrederick 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. ..
- Solid-state NMR methods for investigating native and aggregated eye lens proteinsRACHEL WAGNER MARTIN; Fiscal Year: 2013DESCRIPTION (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...
- Phase boundaries and liquid structure of concentrated eye lens protein mixturesGeorge 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 ..
- Gamma crystallin modifications &mechanisms of lens opacityJayanti 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...
- MODIFICATION OF ALPHA-CRYSTALLIN CHAPERONE FUNCTIONEdathara C Abraham; Fiscal Year: 2013..More than half of the muitants occur in crystallins, in [unreadable]A- and [unreadable]B-crystallins mostly...
- ANALYSIS OF THE ENZYMATIC PROPERTIES OF CRYSTALLINWILLIAM 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...
- A CRYSTALLIN MUTATION WITH ABNORMAL ASTROCYTES AND RETINAL VESSELSDebasish 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. ..
- MECHANISM OF SELENIUM INDUCED CATARACTThomas Shearer; Fiscal Year: 2002..protease (m-calpain), loss of the N-terminal extensions on beta-crystallin polypeptides, insolubilization of crystallins, and opacity...
- Notch Signaling and Lens DevelopmentPumin Zhang; Fiscal Year: 2010..We will validate this initial finding with in vitro and in vivo experiments. ..
- LENS PROTEIN GLYCATION AND CATARACT DEVELOPMENTEDATHARA ABRAHAM; Fiscal Year: 1999b>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)...
- Alpha Crystallin Function in the ZebrafishMason Posner; Fiscal Year: 2010DESCRIPTION (provided by applicant): The vertebrate ??crystallins play an essential role in lens development and the maintenance of lens transparency...
- TARGETED ABLATION IN EYE AND BRAINS Potter; Fiscal Year: 1993..The studies will also provide murine model systems for specific developmental defects such s retinitis pigmentosa...
- Alpha-Crystallin and CataractogensisJoseph Horwitz; Fiscal Year: 2009..These are excellent models for understanding the biochemical and biophysical changes that occur in age-related human cataract. ..
- The role of alphaB-crystallin in chemoresistance of retinoblastomaMERIDETH 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]..
- LENS PROTEIN ALPHA-CRYSTALLIN B CHAINJames Goldman; Fiscal Year: 1999..2. further define how aB-crystallinprotects cells from hypertonic and other stresses, 3. explore mechanisms of Rosenthal Fiber production. ..
- Role of Deamidation in Human Beta-Crystallin StructureKIRSTEN 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...
- Beta-B1 Crystallin - a New Candidate Uveitis AutoantigenLynn Gordon; Fiscal Year: 2003..abstract_text> ..
- PROTEIN GLYCATION--STRUCTURE AND STABILITY OF PRODUCTSAjay Pande; Fiscal Year: 1993..Among the lens crystallins, the gamma-crystallins are maximally glycated, both in vivo and in vitro...