Kutzner Carl Elias, Bauer Karen Carolyn, Hoppe Thorsten
Institute for Genetics, University of Cologne, Cologne, Germany.
Cologne Excellence Cluster on Cellular Stress Responses in Aging Associated Diseases (CECAD), Cologne, Germany.
MicroPubl Biol. 2023 Apr 21;2023. doi: 10.17912/micropub.biology.000824. eCollection 2023.
Muscle is a highly dynamic tissue in which a variety of folding and degradation processes are active to maintain protein homeostasis (proteostasis) and functionality. The muscle-specific chaperone UNC-45 folds the motor protein myosin and assembles it into myofilaments. Malfunction of this chaperone leads to misfolding of myosin, disorganization of myofilaments, and degradation of misfolded myosin molecules by the proteasome. Here, we present a new muscle-specific ubiquitin fusion degradation (UFD) model substrate in that helps clarify how UNC-45 dysfunction affects muscle proteostasis.
肌肉是一种高度动态的组织，其中各种折叠和降解过程活跃，以维持蛋白质稳态（蛋白平衡）和功能。肌肉特异性分子伴侣UNC-45折叠运动蛋白肌球蛋白并将其组装成肌丝。这种分子伴侣的功能障碍会导致肌球蛋白错误折叠、肌丝紊乱，以及蛋白酶体对错误折叠的肌球蛋白分子进行降解。在此，我们提出了一种新的肌肉特异性泛素融合降解（UFD）模型底物，它有助于阐明UNC-45功能障碍如何影响肌肉蛋白平衡。
