Protein Domain : Tim10-like IPR004217

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

0 Child Features

4 Gene Families

647 Genes

0 Ontology Annotations

0 Parent Features

14 Publications

USDA
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