TIMM13
Description
The TIMM13 (translocase of inner mitochondrial membrane 13) is a protein-coding gene located on chromosome 19.
The TIMM13 gene encodes a translocase, similar to yeast mitochondrial proteins, that participates in the import of metabolite transporters from the cytoplasm into the mitochondrial inner membrane. The encoded protein, along with TIMM8a, forms a 70 kDa complex in the intermembrane space. This gene is situated next to the gene for lamin B2, with a head-to-tail orientation.
TIMM13, a mitochondrial intermembrane chaperone, assists in the import and insertion of specific multi-pass transmembrane proteins into the mitochondrial inner membrane. It also plays a crucial role in transferring beta-barrel precursors from the TOM complex to the SAM complex of the outer membrane. Acting as a chaperone-like protein, TIMM13 safeguards hydrophobic precursors from aggregation and guides them through the intermembrane space. The TIMM8-TIMM13 complex facilitates the import of proteins like TIMM23, SLC25A12/ARALAR1, and SLC25A13/ARALAR2. In contrast, the predominant TIMM9-TIMM10 70 kDa complex handles the import of a wider range of proteins.
TIMM13 is also known as TIM13, TIM13B, TIMM13A, TIMM13B, ppv1.
Associated Diseases
- hepatorenocardiac degenerative fibrosis
- Mayer-Rokitansky-Kuster-Hauser syndrome
- 46,XX ovotesticular disorder of sex development
- testicular agenesis
- nephronophthisis
- Senior-Boichis syndrome
- nephronophthisis 3
- cancer
- 46,XY sex reversal 3
