人 TIMM13 抗体对
01AB03292
Translocase of inner mitochondrial membrane 13 (TIMM13) is a mitochondrial intermembrane space chaperone protein essential for the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane, functioning as part of a 70 kDa complex with TIMM8A. TIMM13 collects hydrophobic precursor proteins from the translocase of the outer membrane (TOM) complex and delivers them to either the sorting and assembly machinery (SAM) in the outer membrane or the TIMM22 complex in the inner membrane, thereby preventing aggregation and ensuring proper mitochondrial protein biogenesis. This process is crucial for mitochondrial integrity and function, as TIMM13 is required for the assembly of key proteins such as TIMM23, SLC25A12, and SLC25A13. Mutations or dysfunction in TIMM13 or its complex can lead to mitochondrial dysfunction and are associated with diseases such as Mohr-Tranebjaerg syndrome, a rare neurodegenerative disorder characterized by hearing loss and dystonia, and have been implicated in visual cortex disease.
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技术参数
- 靶点基因名
- TIMM13
- 基因别名
- TIM13B; TIMM13A; TIMM13B
- 蛋白别名
- translocase of inner mitochondrial membrane 13; TIMM13
- UniProt
- Q9Y5L4
- 物种
- Human
产品详情
- 靶点名称
- TIMM13
- 蛋白名称
- translocase of inner mitochondrial membrane 13
- 蛋白别名
- Mitochondrial import inner membrane translocase subunit Tim13
- 靶点基因名
- TIMM13
- 基因别名
- TIM13B; TIMM13A; TIMM13B
- UniProt
- Q9Y5L4
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Translocase of inner mitochondrial membrane 13 (TIMM13) is a mitochondrial intermembrane space chaperone protein essential for the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane, functioning as part of a 70 kDa complex with TIMM8A. TIMM13 collects hydrophobic precursor proteins from the translocase of the outer membrane (TOM) complex and delivers them to either the sorting and assembly machinery (SAM) in the outer membrane or the TIMM22 complex in the inner membrane, thereby preventing aggregation and ensuring proper mitochondrial protein biogenesis. This process is crucial for mitochondrial integrity and function, as TIMM13 is required for the assembly of key proteins such as TIMM23, SLC25A12, and SLC25A13. Mutations or dysfunction in TIMM13 or its complex can lead to mitochondrial dysfunction and are associated with diseases such as Mohr-Tranebjaerg syndrome, a rare neurodegenerative disorder characterized by hearing loss and dystonia, and have been implicated in visual cortex disease.