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- bs-11769P
- 2025年10月16日
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500ug
| 产品编号 | bs-11769P |
| 英文名称 | TIMM8A Antibody Blocking Peptide |
| 中文名称 | 线粒体内膜转位酶8A/耳聋/肌张力障碍肽封闭多肽 |
| 英文别名 | DDP 1; DDP; DDP1; Deafness dystonia protein 1; Deafness/dystonia peptide; DFN 1; DFN1; MGC12262; Mitochondrial import inner membrane translocase subunit Tim8 A; MTS; TIM 8A; TIM8A; TIMM 8A; Translocase of inner mitochondrial membrane 8 homolog A; X linked deafness dystonia protein; TIM8A_HUMAN. |
| 纯化方法 | HPLC |
| 研究领域 | Neuroscience > Neurology process > Neurodegenerative disease Neuroscience > Neurology process > Neurogenesis Neuroscience > Sensory System > Auditory system Signal Transduction > Protein Trafficking > Organelle Proteins |
| 亚基 | Heterohexamer; composed of 3 copies of TIMM8A and 3 copies of TIMM13, named soluble 70 kDa complex. Associates with the TIM22 complex, whose core is composed of TIMM22. |
| 亚细胞定位 | Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side. |
| 组织特异性 | Highly expressed in fetal and adult brain, followed by fetal lung, liver and kidney. Also expressed in heart, placenta, lung, liver, kidney, pancreas, skeletal muscle and heart. |
| 相似性 | Belongs to the small Tim family. |
| 功能 | TIMM8A is a mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins, such as metabolite transporters, from the cytoplasm into the mitochondrial inner membrane. The TIMM8A gene is mutated in Deafness Dystonia Syndrome (MTS/DFN-1) suggesting that it is required for normal neurologic development. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | The majority of mitochondrial-directed proteins are encoded by the nuclear genome and are transported to the mitochondria via regulated processes involving the mitochondrial Tom and Tim proteins (1). The mitochondrial Tim protein family is comprised of a large group of evolutionarily conserved proteins that are found in most eukaryotes (1,2). Import of nuclear-encoded precursor proteins into and across the mitochondrial inner membrane is mediated by two distinct complexes, the Tim23 complex and the Tim22 complex, which differ in their substrate specificity (1). Defects in Tim proteins are implicated in several neuro-degenerative diseases, suggesting important roles for Tim proteins in development and health (3,4). Tim8A and Tim8B, which map to human chromosomes Xq22.1 and 11q23.1-q23.2, respectively, are conserved proteins of the mitochondrial intermembrane space, which are organized in hetero-oligomeric complex with Tim13 (5,6,7). Tim8A is highly expressed in fetal and adult brain (5). Tim8A is mutated in deafness dystonia syndrome, a novel type of disease that causes severe neurological defects, thought to be caused by a defective mitochondrial protein transport system (5,8). |
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TIMM8A Antibody Blocking Peptide(bs-11769P)-500ug
￥880
