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Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins. Probably necessary for normal neurologic development.
Gene Name: | translocase of inner mitochondrial membrane 8 homolog A (yeast) |
Synonyms: | TIMM8A, DDP1, Deafness dystonia protein 1, Deafness/dystonia peptide, DFN1, MTS, DDP, TIM8, TIM8A |
Target Sequences: | NM_004085 NP_004076.1 O60220 |
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