Imbalances in protein homeostasis caused by mutant desmin

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. Me...

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Veröffentlicht in:	Neuropathology and applied neurobiology 2019-08, Vol.45 (5), p.476-494
Hauptverfasser:	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.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animal models Autophagy Crystallin Cytoskeleton Desmin desminopathy Electron microscopy Heat shock proteins Homeostasis Hsp27 protein Hsp90 protein immortalized myoblasts Immunoblotting Immunofluorescence Microscopy Musculoskeletal system Myoblasts Myotubes p53 Protein Phagocytosis Proteasomes protein homeostasis protein quality control Proteins Quality control R349P desmin knock‐in mice Skeletal muscle
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