CHCHD3
Function
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 (PubMed:25781180, PubMed:32567732, PubMed:33130824). Plays an important role in the maintenance of the MICOS complex stability and the mitochondrial cristae morphology (PubMed:25781180, PubMed:32567732, PubMed:33130824). Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription (PubMed:22567091).
Sequence Similarities
Belongs to the MICOS complex subunit Mic19 family. Metazoan Mic19 subfamily.
Tissue Specificity
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.
Cellular localization
- Mitochondrion inner membrane
- Lipid-anchor
- Intermembrane side
- Cytoplasm
- Nucleus
- Mitochondrion
Alternative names
MIC19, MINOS3, CHCHD3, MICOS complex subunit MIC19, Coiled-coil-helix-coiled-coil-helix domain-containing protein 3