TIMM8B

This gene encodes a member of a well-conserved family of proteins with similarity to yeast Tim mitochondrial import proteins. This gene is encoded by a nuclear gene and is transported into the intermembrane space of the mitochondrion. When formed into complexes, these proteins guide membrane-spanning proteins across the mitochondrial intermembrane space before they are added into the mitochondrial inner membrane. This gene is adjacent to succinate dehydrogenase, subunit D (SDHD), in which mutations have been found in affected members of families with hereditary paraganglioma

Full Name

translocase of inner mitochondrial membrane 8 homolog B (yeast)

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).

Biological Process

Biological Process protein insertion into mitochondrial inner membraneSource:FlyBase
Biological Process protein targeting to mitochondrionSource:ProtInc1 Publication
Biological Process sensory perception of soundSource:ProtInc1 Publication

Cellular Location

Mitochondrion inner membrane