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Article

Endoplasmic reticulum stress-induced caspase-4 activation mediates apoptosis and neurodegeneration in INCL

27 Apr 2006

Abstract excerpt

Infantile neuronal ceroid lipofuscinosis (INCL), a neurodegenerative storage disorder of childhood, is caused by mutations in the palmitoyl-protein thioesterase-1 (PPT1) gene. PPT1 cleaves thioester linkages in S-acylated (palmitoylated) proteins and its mutation causes abnormal intracellular accumulation of fatty-acylated proteins and peptides leading to INCL pathogenesis. Although apoptosis is the suggested...

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PMID: 16644870
DOI: 10.1093/hmg/ddl105

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Endoplasmic reticulum stress-induced caspase-4 activation mediates apoptosis and neurodegeneration in INCLPMID 16644870Read ↗

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