Renal Division, Department of Medicine, Emory University, Atlanta, Georgia 30322, USA.
J Am Soc Nephrol. 2010 Jul;21(7):1174-83. doi: 10.1681/ASN.2009101011. Epub 2010 Apr 29.
X-chromosome-linked inhibitor of apoptosis protein (XIAP) is an endogenous caspase inhibitor. Caspase-3 contributes to the muscle wasting associated with chronic kidney disease (CKD) and other systemic illnesses, but whether XIAP modulates muscle wasting in CKD is unknown. Here, overexpression of XIAP in cultured skeletal muscle cells decreased protein degradation induced by serum deprivation, suggesting that caspase-mediated proteolysis contributes to muscle atrophy. We generated transgenic mice that overexpress human XIAP specifically in skeletal muscle (mXIAP) and evaluated muscle protein degradation induced by CKD. mXIAP mice with normal kidney function exhibited mild skeletal muscle hypertrophy. Muscle weights of mXIAP mice with CKD (mXIAP-CKD) were indistinguishable from wild-type mice, suggesting that overexpression of XIAP in skeletal muscle protects from CKD-induced muscle atrophy. The rate of total protein degradation, proteasome chymotrypsin-like activity, and caspase-3-mediated actin cleavage all were lower in muscle isolated from mXIAP-CKD mice compared with wild-type CKD mice. Concomitant with the reduction in overall proteolysis, mRNA levels of ubiquitin, muscle-specific ring finger 1, and atrogin-1/muscle atrophy F-box were lower in mXIAP-CKD mice, suggesting that decreased expression of the ubiquitin-proteasome pathway components may contribute to the protein-sparing effects of XIAP. In summary, these results demonstrate that XIAP inhibits multiple aspects of protein degradation in skeletal muscle during CKD.
X 染色体连锁凋亡蛋白抑制因子(XIAP)是一种内源性半胱天冬酶抑制剂。半胱天冬酶-3 参与与慢性肾脏病(CKD)和其他全身性疾病相关的肌肉消耗,但 XIAP 是否调节 CKD 中的肌肉消耗尚不清楚。在这里,XIAP 在培养的骨骼肌细胞中的过表达减少了血清剥夺诱导的蛋白质降解,这表明半胱天冬酶介导的蛋白水解有助于肌肉萎缩。我们生成了在骨骼肌中特异性过表达人 XIAP 的转基因小鼠(mXIAP),并评估了 CKD 诱导的肌肉蛋白降解。肾功能正常的 mXIAP 小鼠表现出轻微的骨骼肌肥大。CKD 时 mXIAP 小鼠(mXIAP-CKD)的肌肉重量与野生型小鼠无差异,表明骨骼肌中 XIAP 的过表达可防止 CKD 引起的肌肉萎缩。与野生型 CKD 小鼠相比,从 mXIAP-CKD 小鼠分离的肌肉中总蛋白质降解率、蛋白酶体糜蛋白酶样活性和 caspase-3 介导的肌动蛋白切割均降低。伴随着整体蛋白水解的减少,mXIAP-CKD 小鼠的肌肉中泛素、肌肉特异性环指 1 和 atrogin-1/肌肉萎缩 F 盒的 mRNA 水平降低,这表明泛素-蛋白酶体途径成分表达减少可能有助于 XIAP 的蛋白保护作用。总之,这些结果表明 XIAP 在 CKD 期间抑制骨骼肌中多种蛋白质降解途径。