PSMC1 - proteasome 26S subunit, ATPase 1 Gene

中文名称：蛋白酶体 26S 亚基，ATPase 1

种属: Homo sapiens

同用名: S4; p56; RPT2; P26S4; NEDGTH

关于 PSMC1

功能概要

26S 蛋白酶体是一种多催化蛋白酶复合物，具有由 2 个复合物、20S 核心和 19S 调节剂组成的高度有序结构。 20S 核心由 28 个不同亚基的 4 个环组成； 2 个环由 7 个α亚基组成，2 个环由 7 个β亚基组成。 19S 调节器由一个包含 6 个 ATP 酶亚基和 2 个非 ATP 酶亚基的碱基和一个包含多达 10 个非 ATP 酶亚基的盖子组成。蛋白酶体以高浓度分布在整个真核细胞中，并在非溶酶体途径中以 ATP/泛素依赖性过程切割肽。修饰的蛋白酶体 (免疫蛋白酶体) 的基本功能是加工 I 类 MHC 肽。该基因编码一个 ATP 酶亚基，它是具有伴侣样活性的 ATP 酶三 A 家族的成员。该亚基和一个 20S 核心 α 亚基与乙型肝炎病毒 X 蛋白 (一种对病毒复制至关重要的蛋白) 特异性相互作用。该亚基还与腺病毒 E1A 蛋白相互作用，这种相互作用会改变蛋白酶体的活性。最后，该亚基与 ataxin-7 相互作用，表明蛋白酶体在 7 型脊髓小脑性共济失调 (一种进行性神经退行性疾病) 的发展中发挥作用。[RefSeq 提供，2008 年 7 月]

The 26S Proteasome is a multicatalytic proteinase complex with a highly ordered structure composed of 2 complexes, a 20S core and a 19S regulator. The 20S core is composed of 4 rings of 28 non-identical subunits; 2 rings are composed of 7 alpha subunits and 2 rings are composed of 7 beta subunits. The 19S regulator is composed of a base, which contains 6 ATPase subunits and 2 non-ATPase subunits, and a lid, which contains up to 10 non-ATPase subunits. Proteasomes are distributed throughout eukaryotic cells at a high concentration and cleave Peptides in an ATP/ubiquitin-dependent process in a non-lysosomal pathway. An essential function of a modified Proteasome, the immunoproteasome, is the processing of class I MHC Peptides. This gene encodes one of the ATPase subunits, a member of the triple-A family of ATPases which have a chaperone-like activity. This subunit and a 20S core alpha subunit interact specifically with the hepatitis B virus X protein, a protein critical to viral replication. This subunit also interacts with the adenovirus E1A protein and this interaction alters the activity of the Proteasome. Finally, this subunit interacts with ataxin-7, suggesting a role for the Proteasome in the development of spinocerebellar ataxia type 7, a progressive neurodegenerative disorder. [provided by RefSeq, Jul 2008]