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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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: Deafness dystonia protein 1; deafness/dystonia peptide; Mitochondrial import inner membrane translocase subunit Tim8 A; X-linked deafness dystonia protein
Gene Aliases: DDP; DDP1; DFN1; MTS; TIM8; TIM8A; TIMM8A
UniProt ID: (Human) O60220
Entrez Gene ID: (Human) 1678
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