内质网应激和 ERO1：遗传性肌病的潜在治疗靶点。
ER stress and ERO1: Potential therapeutic targets for inherited myopathies.
机构信息
Department of Pharmacological and Pharmaceutical Sciences, University of Houston College of Pharmacy, Houston, TX 77204, USA.
Department of Pharmacological and Pharmaceutical Sciences, University of Houston College of Pharmacy, Houston, TX 77204, USA.
出版信息
Cell Rep Med. 2024 Mar 19;5(3):101462. doi: 10.1016/j.xcrm.2024.101462.
Abstract
Selenoprotein N-related myopathy (SEPN1-RM) is a genetic disease that causes muscle weakness and respiratory failure. Germani et al. demonstrate that diaphragm weakness in SEPN1-RM is prevented by the inhibition of ER stress or ERO1 oxidoreductase regulated by transcription factor CHOP.
摘要
硒蛋白 N 相关肌病（SEPN1-RM）是一种遗传性疾病，可导致肌肉无力和呼吸衰竭。Germani 等人证明，通过抑制内质网应激或 CHOP 转录因子调节的 ERO1 氧化还原酶，可预防 SEPN1-RM 的膈肌无力。
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