CHCHD3 Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription. Plays an important role in the maintenance of the MICOS complex stability and the mitochondrial cristae morphology. Belongs to the MICOS complex subunit Mic19 family. Metazoan Mic19 subfamily. Detected at low levels in brain, placenta, lung, liver, kidney and pancreas with increased levels in heart and skeletal muscle. Higher expression in primary lung cancers than in normal lung tissue. Note: This description may include information from UniProtKB.
Protein type: Mitochondrial
Chromosomal Location of Human Ortholog: 6|6 A3.3
Cellular Component:  cytoplasm; membrane; MICOS complex; mitochondrial inner membrane; mitochondrion; nucleus
Molecular Function:  DNA-binding transcription repressor activity, RNA polymerase II-specific; molecular adaptor activity; phosphatase binding; protein binding
Biological Process:  cristae formation; inner mitochondrial membrane organization; mitochondrial fusion; negative regulation of transcription by RNA polymerase II
Reference #:  Q9CRB9 (UniProtKB)
Alt. Names/Synonyms: 0610041L09Rik; 1700039J09Rik; AW558177; CHCH3; Chchd3; coiled-coil-helix-coiled-coil-helix domain containing 3; Coiled-coil-helix-coiled-coil-helix domain-containing protein 3, mitochondrial; MICOS complex subunit Mic19; OTTMUSP00000015630
Gene Symbols: Chchd3
Molecular weight: 26,335 Da
Basal Isoelectric point: 8.56  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