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CLPB Q9H078

线粒体解聚酶 (EC 3.6.1.-) (钾转运缺陷抑制因子3) [裂解为:线粒体解聚酶,裂解形式]

蛋白质信息 (UniProt)

线粒体解聚酶 (EC 3.6.1.-) (钾转运缺陷抑制因子3) [裂解为:线粒体解聚酶,裂解形式]

Q9H078

功能描述

作为调节性ATPase发挥作用,参与分泌/蛋白质运输过程。具有ATP依赖性蛋白质解聚酶活性,是维持关键线粒体蛋白质可溶性所必需的 (PubMed:32573439, PubMed:34115842, PubMed:35247700, PubMed:36170828, PubMed:36745679)。参与线粒体介导的抗病毒先天免疫,激活RIG-I介导的信号转导以及IFNB1和促炎细胞因子IL6的产生 (PubMed:31522117)。在粒细胞分化中发挥作用 (PubMed:34115842)。 {ECO:0000269|PubMed:31522117, ECO:0000269|PubMed:32573439, ECO:0000269|PubMed:34115842, ECO:0000269|PubMed:35247700, ECO:0000269|PubMed:36170828, ECO:0000269|PubMed:36745679}。

组织特异性

Widely expressed (at protein level) (PubMed:25597511). Expressed in fetal, as well as in adult tissues, with highest levels in adult brain, including thalamus, hippocampus, occipital cortex and parietal cortex. Low expression in granulocytes (PubMed:25597510).

亚细胞定位

Mitochondrion intermembrane space

关键词

3D-structure Acetylation Alternative splicing ANK repeat ATP-binding Cataract Disease variant Epilepsy Hydrolase Intellectual disability