TTC19 Mitochondrial protein required for formation of the mitochondrial complex III (PubMed:21278747). May also be required for the abcission step in cytokinesis, possibly regulating the ESCRT-III complex via its interaction with CHMP4B (PubMed:20208530). However, the involvement in cytokinesis requires additional experimental evidence. Defects in TTC19 are a cause of mitochondrial complex III deficiency (MT-C3D). A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. Belongs to the TTC19 family. Note: This description may include information from UniProtKB.
Protein type: Mitochondrial
Chromosomal Location of Human Ortholog: 11|11 B2
Cellular Component:  membrane; mitochondrial inner membrane; mitochondrion; respiratory chain
Molecular Function:  protein binding
Biological Process:  mitochondrial respiratory chain complex III assembly; oxidation-reduction process
Reference #:  Q8CC21 (UniProtKB)
Alt. Names/Synonyms: 2010204O13Rik; 2810460C24Rik; AI505442; OTTMUSP00000006049; tetratricopeptide repeat domain 19; Tetratricopeptide repeat protein 19; Tetratricopeptide repeat protein 19, mitochondrial; TPR repeat protein 19; Ttc19
Gene Symbols: Ttc19
Molecular weight: 41,234 Da
Basal Isoelectric point: 5.87  Predict pI for various phosphorylation states
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Protein Structure Not Found.

Cross-references to other databases:  STRING  |  BioGPS  |  Pfam  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene