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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
技术规格
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Full name: |
translocase of inner mitochondrial membrane 8A |
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Synonyms: |
DDP; MTS; DDP1; DFN1; TIM8 |
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Swissprot: |
O60220 |
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Gene Accession: |
BC006994 |
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Purity: |
>85%, as determined by Coomassie blue stained SDS-PAGE |
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Expression system: |
Escherichia coli |
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Tags: |
His tag C-Terminus, GST tag N-Terminus |
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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. |
