DnaJ同源亚家族C成员30，线粒体（Williams-Beuren综合征染色体区域18蛋白）
DnaJ同源亚家族C成员30，线粒体（Williams-Beuren综合征染色体区域18蛋白）
Q96LL9功能描述
富集于神经元的线粒体蛋白，作为线粒体呼吸的调节因子 (By similarity)。与ATP合酶复合物结合并促进ATP合成 (By similarity)。可能是一种参与线粒体复合物I N模块亚基周转的伴侣蛋白。它促进被氧化应激损伤的N模块亚基的降解，并有助于复合物I的功能效率 (PubMed:33465056)。 {ECO:0000250|UniProtKB:P59041, ECO:0000269|PubMed:33465056}.
组织特异性
Expressed in brain, heart, kidney, liver, lung, spleen, stomach and testis (PubMed:12073013). Highly expressed in the brain (PubMed:30318146). In the neocortex, expressed in most, if not all, glutamatergic excitatory projection neurons (pyramidal) and many interneurons, with the strongest signal noticeably in large pyramidal neurons of layer 3C. Also present in pyramidal neurons of layer 3C PNs of the superior temporal cortex, as well as in pyramidal neurons (Betz cells) of the layer 5B primary motor cortex (at protein level) (PubMed:30318146).
亚细胞定位
Mitochondrion inner membrane
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