TMEM126A Q9H061

跨膜蛋白126A

功能描述

线粒体内膜中共翻译蛋白质质量控制所需的蛋白质 (PubMed:38199007)。与新合成的多肽结合，可能作为分子伴侣与OXA1L协作，将新合成的线粒体蛋白质插入内膜 (PubMed:38199007)。线粒体复合物I ND4模块组装所必需的 (PubMed:33879611, PubMed:33882309)。 {ECO:0000269|PubMed:33879611, ECO:0000269|PubMed:33882309, ECO:0000269|PubMed:38199007}。

组织特异性

Strongly expressed in brain, cerebellum, skeletal muscle, testis. High expression also found in fetal brain, fetal retinal pigmentary epithelium, and fetal retina. Highly expressed in retinal ganglion cells.

亚细胞定位

Mitochondrion inner membrane