Protein: Mitochondrial import inner membrane translocase subunit Tim8 A (Deafness dystonia protein 1) (X-linked deafness dystonia protein) (O60220)
Organism: Homo sapiens (Human)
Function: 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.
Subcellular location: Mitochondrion inner membrane {ECO:0000269|PubMed:11489896}; Peripheral membrane protein {ECO:0000269|PubMed:11489896}; Intermembrane side {ECO:0000269|PubMed:11489896}.
Sequence: MDSSSSSSAAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
InterPro domains: - IPR035427: Tim10-like domain superfamily (homologous_superfamily) [23-85]
- IPR004217: Tim10-like (domain) [22-83]
Interaction partners: - Mitochondrial import inner membrane translocase subunit Tim13
- Mitochondrial import inner membrane translocase subunit Tim10
- Mitochondrial import inner membrane translocase subunit Tim9
- Mitochondrial import inner membrane translocase subunit Tim10 B
- Mitochondrial intermembrane space import and assembly protein 40
- Mitochondrial import inner membrane translocase subunit Tim23
- Electrogenic aspartate/glutamate antiporter SLC25A12, mitochondrial
- Electrogenic aspartate/glutamate antiporter SLC25A13, mitochondrial
- Mitochondrial import inner membrane translocase subunit Tim22
- Mitochondrial import receptor subunit TOM40 homolog
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0043167 (ion binding), GO:0005515 (protein binding), GO:0043169 (cation binding), GO:0046872 (metal ion binding), GO:0046914 (transition metal ion binding), GO:0008270 (zinc ion binding)
Biological Process (BP): GO:0008150 (biological process), GO:0051179 (localization), GO:0009987 (cellular process), GO:0032501 (multicellular organismal process), GO:0051641 (cellular localization), GO:0051234 (establishment of localization), GO:0071840 (cellular component organization or biogenesis), GO:0003008 (system process), GO:0033036 (macromolecule localization), GO:0046907 (intracellular transport), GO:0006810 (transport), GO:0070727 (cellular macromolecule localization), GO:0051649 (establishment of localization in cell), GO:0016043 (cellular component organization), GO:0050877 (nervous system process), GO:0051668 (localization within membrane), GO:0045184 (establishment of protein localization), GO:0006839 (mitochondrial transport), GO:0090151 (establishment of protein localization to mitochondrial membrane), GO:0006886 (intracellular protein transport), GO:0071705 (nitrogen compound transport), GO:0015031 (protein transport), GO:0072594 (establishment of protein localization to organelle), GO:0071702 (organic substance transport), GO:0008104 (protein localization), GO:0006996 (organelle organization), GO:0007600 (sensory perception), GO:0090150 (establishment of protein localization to membrane), GO:0061024 (membrane organization), GO:0072657 (protein localization to membrane), GO:0072655 (establishment of protein localization to mitochondrion), GO:0007005 (mitochondrion organization), GO:0033365 (protein localization to organelle), GO:0007006 (mitochondrial membrane organization), GO:0006605 (protein targeting), GO:0051205 (protein insertion into membrane), GO:0050954 (sensory perception of mechanical stimulus), GO:0051204 (protein insertion into mitochondrial membrane), GO:0006626 (protein targeting to mitochondrion), GO:0070585 (protein localization to mitochondrion), GO:0007605 (sensory perception of sound), GO:0007007 (inner mitochondrial membrane organization), GO:0045039 (protein insertion into mitochondrial inner membrane)
Cellular Component (CC): GO:0005575 (cellular component), GO:0032991 (protein-containing complex), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0098798 (mitochondrial protein-containing complex), GO:0016020 (membrane), GO:0098796 (membrane protein complex), GO:1990351 (transporter complex), GO:0031975 (envelope), GO:0005737 (cytoplasm), GO:0031974 (membrane-enclosed lumen), GO:0031090 (organelle membrane), GO:0031967 (organelle envelope), GO:0043229 (intracellular organelle), GO:0098800 (inner mitochondrial membrane protein complex), GO:0005739 (mitochondrion), GO:0043233 (organelle lumen), GO:0043227 (membrane-bounded organelle), GO:0005740 (mitochondrial envelope), GO:0043231 (intracellular membrane-bounded organelle), GO:0031970 (organelle envelope lumen), GO:0019866 (organelle inner membrane), GO:0070013 (intracellular organelle lumen), GO:0031966 (mitochondrial membrane), GO:0005758 (mitochondrial intermembrane space), GO:0005743 (mitochondrial inner membrane)