Structural basis for mutation-induced destabilization of profilin 1 in ALS
نویسندگان
چکیده
منابع مشابه
Structural basis for mutation-induced destabilization of profilin 1 in ALS.
Mutations in profilin 1 (PFN1) are associated with amyotrophic lateral sclerosis (ALS); however, the pathological mechanism of PFN1 in this fatal disease is unknown. We demonstrate that ALS-linked mutations severely destabilize the native conformation of PFN1 in vitro and cause accelerated turnover of the PFN1 protein in cells. This mutation-induced destabilization can account for the high prop...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2015
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.1424108112