TIMM8B
Domain
The twin CX3C motif contains 4 conserved Cys residues that form 2 disulfide bonds in the mitochondrial intermembrane space. However, during the transit of TIMM8B from cytoplasm into mitochondrion, the Cys residues probably coordinate zinc, thereby preventing folding and allowing its transfer across mitochondrial outer membrane (By similarity).
Function
Probable 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 (By similarity).
Sequence Similarities
Belongs to the small Tim family.
Tissue Specificity
Ubiquitous, with highest expression in heart, kidney, liver and skeletal muscle.
Cellular localization
- Mitochondrion inner membrane
- Peripheral membrane protein
- Intermembrane side
Alternative names
DDP2, DDPL, TIM8B, TIMM8B, Mitochondrial import inner membrane translocase subunit Tim8 B, DDP-like protein, Deafness dystonia protein 2