Domain

Tim10-like (IPR004217)

Short name: Tim10-like

Domain relationships

None.

Description

This domain has four conserved cysteine residues. It is found in proteins Tim8, Tim9, Tim10 and Tim13, which are involved in mitochondrial protein import [PMID: 11101512] and seem to be localised to the mitochondrial intermembrane space. The Tim8-Tim13 complex has a complex architecture, similar to the Tim9-Tim10 complex, composed of a hexametric architecture with long helices which look like tentacles extend from a central loop [PMID: 18706423]. In each subunit of the Tim9-Tim10 complex, a signature "twin CX3C" motif forms two intramolecular disulfides [PMID: 16387659].

Defects in the Tim8A gene (DDP1) have been shown to be the cause of 2 human syndromes: Mohr-Tranebjaerg syndrome (MTS); also known as dystonia-deafness syndrome (DDS) or X-linked progressive deafness type 1 (DFN-1); and Jensen syndrome (JENSS); also known as opticoacoustic nerve atrophy with dementia.

Contributing signatures

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