Name: TIMM8A 抗原（重组蛋白）
Brand: 通蔚生物

中文名称：TIMM8A 抗原（重组蛋白）

英文名称：TIMM8A Antigen (Recombinant Protein)

别名： translocase of inner mitochondrial membrane 8A; DDP; MTS; DDP1; DFN1; TIM8

储存： 冷冻（-20℃）

相关类别：抗原

概述

Fusion protein corresponding to a region derived from 1-97 amino acids of human TIMM8A

技术规格

Full name:	translocase of inner mitochondrial membrane 8A
Synonyms:	DDP; MTS; DDP1; DFN1; TIM8
Swissprot:	O60220
Gene Accession:	BC006994
Purity:	>85%, as determined by Coomassie blue stained SDS-PAGE
Expression system:	Escherichia coli
Tags:	His tag C-Terminus, GST tag N-Terminus
Background:	This translocase is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in this gene also cause Jensen syndrome; an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. This protein, along with TIMM13, forms a 70 kDa heterohexamer. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

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