TIMM8A : translocase of inner mitochondrial membrane 8A
Description
The TIMM8A (translocase of inner mitochondrial membrane 8A) is a protein-coding gene located on chromosome X.
The TIMM8A gene provides instructions for making a protein found inside mitochondria. Mitochondria, the powerhouses of cells, have two membranes: an outer and an inner membrane, separated by the intermembrane space. TIMM8A resides in this space, forming a complex with a similar protein called TIMM13. This complex helps transport other proteins across the intermembrane space to the inner mitochondrial membrane.
TIMM8A is a mitochondrial intermembrane chaperone that plays a critical role in importing and inserting multi-pass transmembrane proteins into the mitochondrial inner membrane. It also facilitates the transfer of beta-barrel precursors from the TOM complex to the SAM complex in the outer membrane. Acting as a chaperone, TIMM8A protects hydrophobic precursors from aggregation and guides them through the intermembrane space. TIMM8A forms a complex with TIMM13, known as the TIMM8-TIMM13 complex, which mediates the import of specific proteins like TIMM23, SLC25A12/ARALAR1, and SLC25A13/ARALAR2. While the TIMM9-TIMM10 complex handles the import of a wider range of proteins, TIMM8A's role in the import of these specific proteins is crucial. TIMM8A is likely essential for proper neurologic development.
TIMM8A is also known as DDP, DDP1, DFN1, MTS, TIM8.
