v5.1.0.3
Glycine data from LIS
Type | Domain |
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 [ ] 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 []. In each subunit of the Tim9-Tim10 complex, a signature "twin CX3C"motif forms two intramolecular disulfides [ ].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. |
Short Name | Tim10-like |