Palmitoylation-induced Aggregation of Cysteine-string Protein Mutants That Cause Neuronal Ceroid Lipofuscinosis

Recently, mutations in the DNAJC5 gene encoding cysteine-string protein α (CSPα) were identified to cause the neurodegenerative disorder adult-onset neuronal ceroid lipofuscinosis. The disease-causing mutations (L115R or ΔL116) occur within the cysteine-string domain, a region of the protein that is...

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Bibliographic Details
Main Authors: Greaves, Jennifer, Lemonidis, Kimon, Gorleku, Oforiwa A., Cruchaga, Carlos, Grefen, Christopher, Chamberlain, Luke H.
Format: Artigo
Language:Inglês
Published: American Society for Biochemistry and Molecular Biology 2012
Subjects:
Protein Structure and Folding
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3481330/
https://ncbi.nlm.nih.gov/pubmed/22902780
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M112.389098
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