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- W2128541330 abstract "Tay-Sachs and Sandhoff diseases are lysosomal storage disorders characterized by the absence of beta-hexosaminidase activity and the accumulation of GM2 ganglioside in neurons. In each disorder, a virtually identical course of neurodegeneration begins in infancy and leads to demise generally by 4-6 years of age. Through serial analysis of gene expression (SAGE), we determined gene expression profiles in cerebral cortex from a Tay-Sachs patient, a Sandhoff disease patient and a pediatric control. Examination of genes that showed altered expression in both patients revealed molecular details of the pathophysiology of the disorders relating to neuronal dysfunction and loss. A large fraction of the elevated genes in the patients could be attributed to activated macrophages/microglia and astrocytes, and included class II histocompatability antigens, the pro-inflammatory cytokine osteopontin, complement components, proteinases and inhibitors, galectins, osteonectin/SPARC, and prostaglandin D2 synthase. The results are consistent with a model of neurodegeneration that includes inflammation as a factor leading to the precipitous loss of neurons in individuals with these disorders." @default.
- W2128541330 created "2016-06-24" @default.
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- W2128541330 date "2002-05-16" @default.
- W2128541330 modified "2023-10-13" @default.
- W2128541330 title "Molecular pathophysiology in Tay-Sachs and Sandhoff diseases as revealed by gene expression profiling" @default.
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- W2128541330 doi "https://doi.org/10.1093/hmg/11.11.1343" @default.
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