The R116C mutation in alpha A-crystallin diminishes its protective ability against stress-induced lens epithelial cell apoptosis.
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Cell death triggered by a novel mutation in the alphaA-crystallin gene underlies autosomal dominant cataract linked to chromosome 21qActivation of metallothioneins and alpha-crystallin/sHSPs in human lens epithelial cells by specific metals and the metal content of aging clear human lensesIncreased monomerization of mutant HSPB1 leads to protein hyperactivity in Charcot-Marie-Tooth neuropathyBiochemical characterization and evaluation of a Brugia malayi small heat shock protein as a vaccine against lymphatic filariasisIn vivo substrates of the lens molecular chaperones αA-crystallin and αB-crystallinMechanism of small heat shock protein function in vivo: a knock-in mouse model demonstrates that the R49C mutation in alpha A-crystallin enhances protein insolubility and cell deathRole of the C-terminal extensions of alpha-crystallins. Swapping the C-terminal extension of alpha-crystallin to alphaB-crystallin results in enhanced chaperone activityAlphaA-crystallin R49Cneo mutation influences the architecture of lens fiber cell membranes and causes posterior and nuclear cataracts in mice.αA-Crystallin associates with α6 integrin receptor complexes and regulates cellular signaling.A knock-in mouse model for the R120G mutation of αB-crystallin recapitulates human hereditary myopathy and cataracts.The Hsp27 gene is not required for Drosophila development but its activity is associated with starvation resistanceSmall heat shock proteins in inherited peripheral neuropathies.Alpha-crystallin-derived peptides as therapeutic chaperones.Comparative proteomic analysis identifies age-dependent increases in the abundance of specific proteins after deletion of the small heat shock proteins αA- and αB-crystallin.Differential role of arginine mutations on the structure and functions of α-crystallin.Mechanism of insolubilization by a single-point mutation in alphaA-crystallin linked with hereditary human cataracts.Cloning of crystallin from orange-spotted grouper and characterization of its activity as potential protective agent.Recombinant human alphaA-crystallin can protect the enzymatic activity of CpUDG against thermal inactivation.Barcoding heat shock proteins to human diseases: looking beyond the heat shock responseSmall heat-shock proteins: important players in regulating cellular proteostasis.Photosensitizing Activity of Endogenous Eye Lens Chromophores: An Attempt to Unravel Their Contributions to Photo-Aging and Cataract Disease.Lens Biology and BiochemistryTrimethylamine N-oxide alleviates the severe aggregation and ER stress caused by G98R alphaA-crystallin.A small heat shock/alpha-crystallin protein from encysted Artemia embryos suppresses tubulin denaturation.The anti-apoptotic function of human αA-crystallin is directly related to its chaperone activity.Role of the conserved SRLFDQFFG region of alpha-crystallin, a small heat shock protein. Effect on oligomeric size, subunit exchange, and chaperone-like activity.Probing the changes in gene expression due to α-crystallin mutations in mouse models of hereditary human cataract.Real-time heterogeneous protein-protein interaction between αA-crystallin N-terminal mutants and αB-crystallin using quartz crystal microbalance (QCM).
P2860
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P2860
The R116C mutation in alpha A-crystallin diminishes its protective ability against stress-induced lens epithelial cell apoptosis.
description
2001 nî lūn-bûn
@nan
2001 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年論文
@yue
2001年論文
@zh-hant
2001年論文
@zh-hk
2001年論文
@zh-mo
2001年論文
@zh-tw
2001年论文
@wuu
name
The R116C mutation in alpha A- ...... ens epithelial cell apoptosis.
@ast
The R116C mutation in alpha A- ...... ens epithelial cell apoptosis.
@en
type
label
The R116C mutation in alpha A- ...... ens epithelial cell apoptosis.
@ast
The R116C mutation in alpha A- ...... ens epithelial cell apoptosis.
@en
prefLabel
The R116C mutation in alpha A- ...... ens epithelial cell apoptosis.
@ast
The R116C mutation in alpha A- ...... ens epithelial cell apoptosis.
@en
P2093
P2860
P356
P1476
The R116C mutation in alpha A- ...... ens epithelial cell apoptosis.
@en
P2093
Harendra C Patel
Jing-Hua Xi
Usha P Andley
P2860
P304
10178-10186
P356
10.1074/JBC.M109211200
P407
P577
2001-12-27T00:00:00Z