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Truncating mutations of SPAST associated with hereditary spastic paraplegia indicate greater accumulation and toxicity of the M1 isoform of spastin

The SPAST gene, which produces two isoforms (M1 and M87) of the microtubule-severing protein spastin, is the chief gene mutated in hereditary spastic paraplegia. Haploinsufficiency is a popular explanation for the disease, in part because most of the >200 pathogenic mutations of the gene are trun...

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Bibliografski detalji
Izdano u:Mol Biol Cell
Glavni autori: Solowska, Joanna M., Rao, Anand N., Baas, Peter W.
Format: Artigo
Jezik:Inglês
Izdano: The American Society for Cell Biology 2017
Teme:
Online pristup:https://ncbi.nlm.nih.gov/pmc/articles/PMC5491181/
https://ncbi.nlm.nih.gov/pubmed/28495799
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1091/mbc.E17-01-0047
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