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• W2078525195 endingPage "2266" @default.
• W2078525195 startingPage "2252" @default.
• W2078525195 abstract "Alexander disease is a primary genetic disorder of astrocyte caused by dominant mutations in the astrocyte-specific intermediate filament glial fibrillary acidic protein (GFAP). While most of the disease-causing mutations described to date have been found in the conserved α-helical rod domain, some mutations are found in the C-terminal non-α-helical tail domain. Here, we compare five different mutations (N386I, S393I, S398F, S398Y and D417M14X) located in the C-terminal domain of GFAP on filament assembly properties in vitro and in transiently transfected cultured cells. All the mutations disrupted in vitro filament assembly. The mutations also affected the solubility and promoted filament aggregation of GFAP in transiently transfected MCF7, SW13 and U343MG cells. This correlated with the activation of the p38 stress-activated protein kinase and an increased association with the small heat shock protein (sHSP) chaperone, αB-crystallin. Of the mutants studied, D417M14X GFAP caused the most significant effects both upon filament assembly in vitro and in transiently transfected cells. This mutant also caused extensive filament aggregation coinciding with the sequestration of αB-crystallin and HSP27 as well as inhibition of the proteosome and activation of p38 kinase. Associated with these changes were an activation of caspase 3 and a significant decrease in astrocyte viability. We conclude that some mutations in the C-terminus of GFAP correlate with caspase 3 cleavage and the loss of cell viability, suggesting that these could be contributory factors in the development of Alexander disease." @default.
• W2078525195 created "2016-06-24" @default.
• W2078525195 creator A5006497097 @default.
• W2078525195 creator A5024493735 @default.
• W2078525195 creator A5026977716 @default.
• W2078525195 creator A5086619393 @default.
• W2078525195 creator A5091055322 @default.
• W2078525195 date "2011-10-01" @default.
• W2078525195 modified "2023-10-01" @default.
• W2078525195 title "Alexander disease causing mutations in the C-terminal domain of GFAP are deleterious both to assembly and network formation with the potential to both activate caspase 3 and decrease cell viability" @default.
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• W2078525195 doi "https://doi.org/10.1016/j.yexcr.2011.06.017" @default.
• W2078525195 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4308095" @default.
• W2078525195 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/21756903" @default.
• W2078525195 hasPublicationYear "2011" @default.
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