| 背景信息 |
Tripartite motif containing 63 (TRIM63), also known as MuRF1, is an E3 ubiquitin ligase localized to the Z-line and M-line lattices of myofibrils in striated muscle. It plays a central role in muscle protein catabolism by mediating the ubiquitination and subsequent proteasomal degradation of key structural proteins, including myosin heavy chain, myosin light chain, and muscle-type creatine kinase. TRIM63 expression is significantly upregulated during skeletal muscle atrophy induced by denervation, immobilization, or glucocorticoid treatment. Beyond its role in skeletal muscle, it regulates cardiac troponin I degradation and influences muscle hypertrophy through PKC-mediated signaling. Clinically, TRIM63 is associated with hypertrophic cardiomyopathy, where homozygous or compound heterozygous mutations lead to concentric left ventricular hypertrophy and dysfunction. It is also linked to the pathogenesis of Danon Disease. |