Article
Imbalances in protein homeostasis caused by mutant desmin.
Neuropathology and applied neurobiology - 1 Aug 2019
Winter L, Unger A, Berwanger C, Spörrer M, Türk M, Chevessier F, Strucksberg K-H, Schlötzer-Schrehardt U, Wittig I, Goldmann W H, Marcus K, Linke W A, Clemen C S, Schröder R
Abstract excerpt
AIMS: We investigated newly generated immortalized heterozygous and homozygous R349P desmin knock-in myoblasts in conjunction with the corresponding desminopathy mice as models for desminopathies to analyse major protein quality control processes in response to the presence of R349P mutant desmin. METHODS: We used hetero- and homozygous R349P desmin knock-in mice for analyses and for crossbreeding with p53...
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