Palmitoylation-induced aggregation of cysteine-string protein mutants that cause neuronal ceroid lipofuscinosis

Jennifer Greaves, Kimon Lemonidis, Oforiwa A. Gorleku, Carlos Cruchaga, Christopher Grefen, Luke H. Chamberlain

Research output: Contribution to journalArticlepeer-review

43 Scopus citations

Abstract

Specific mutations in the chaperone protein CSPα cause adult-onset neuronal ceroid lipofuscinosis. Results: These mutants form SDS-resistant aggregates in a palmitoylation-dependent manner in cell lines and brain samples from mutation carriers. Conclusion: Palmitoylation induces disease-causing CSPα mutants to form SDS-resistant aggregates. Significance: Formation of SDS-resistant CSPα aggregates may underlie development of adult-onset neuronal ceroid lipofuscinosis.

Original languageEnglish
Pages (from-to)37330-37339
Number of pages10
JournalJournal of Biological Chemistry
Volume287
Issue number44
DOIs
StatePublished - Oct 26 2012

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