| Tissue Specificity | Highly expressed in fetal and adult brain, followed by fetal lung, liver and kidney. Also expressed in heart, placenta, lung, liver, kidney, pancreas, skeletal muscle and heart. |
| 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. |
| Protein Name | Mitochondrial Import Inner Membrane Translocase Subunit Tim8 ADeafness Dystonia Protein 1X-Linked Deafness Dystonia Protein |
| Database Links | Reactome: R-HSA-1268020 |
| Cellular Localisation | Mitochondrion Inner MembranePeripheral Membrane ProteinIntermembrane Side |
| Alternative Protein Names | Mitochondrial Import Inner Membrane Translocase Subunit Tim8 A proteinDeafness Dystonia Protein 1 proteinX-Linked Deafness Dystonia Protein proteinTIMM8A proteinDDP proteinDDP1 proteinTIM8A protein |
Information sourced from Uniprot.org
