TIMM8A Proteins

Protein Information

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.

Synonyms

DDP; Ddp1; deafness dystonia protein 1; Deafness dystonia protein 1 homolog; deafness/dystonia peptide; DFN1; DXHXS1274E; Fci-12; Mitochondrial import inner membrane translocase subunit Tim8 A; MTS; TIM8; Tim8a; Timm8a; Timm8a1; translocase of inner mitochondrial membrane 8 homolog a; translocase of inner mitochondrial membrane 8 homolog A (yeast); translocase of inner mitochondrial membrane 8 homolog a1; translocase of inner mitochondrial membrane 8 homolog A1 (yeast); translocase of inner mitochondrial membrane 8A1; X-linked deafness dystonia protein