Recombinant Human Mitochondrial import inner membrane translocase subunit Tim8 B (TIMM8B)

Uniprot Id

Q9Y5J9

Target Species

Human

Target Name

TIMM8B

Target Full Name

Mitochondrial import inner membrane translocase subunit Tim8 B

Target 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.

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side.

Target Protein Families

Small Tim family

Target Tissue Specificity

Ubiquitous, with highest expression in heart, kidney, liver and skeletal muscle.

Target Synonyms

TIMM8B; DDP2; DDPL; TIM8B; Mitochondrial import inner membrane translocase subunit Tim8 B; DDP-like protein; Deafness dystonia protein 2

Target Background

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.