Mitochondrial import inner membrane translocase subunit Tim8/13 (IPR039238)

Short name: Tim8/13

Overlapping homologous superfamilies

Family relationships



The mitochondrial import inner membrane translocase is a 70 kDa, heterohexamer complex consisting of three copies of Tim8 (or Tim8A and/or Tim8B) and three copies of Tim13. The subunits are homologous. The complex is located in the mitochondrial intermembrane space [PMID: 11489896]. It is required for the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane and also for transport of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane [PMID: 11489896]. The complex is more selective than the Tim9-Tim10 complex, and substrates include Tim23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2 [PMID: 15254020]. Tim8 and Tim13 are zinc-finger proteins [PMID: 11489896]. Mutations in the human Tim8A gene cause Mohr-Tranebjaerg syndrome which is an X-linked recessive deafness syndrome associated with progressive visual deterioration, dystonia, dementia, and psychiatric abnormalities [PMID: 11803487].

GO terms

Biological Process

GO:0072321 chaperone-mediated protein transport

Molecular Function

No terms assigned in this category.

Cellular Component

GO:0005758 mitochondrial intermembrane space

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.