MitoProteome Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins

MT001101

UniProt Annotations

Entry Information

Gene Name	coiled-coil-helix-coiled-coil-helix domain containing 3
Protein Entry	MIC19_HUMAN
UniProt ID	Q9NX63
Species	Human

Comments

Comment type	Description
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. Has also been shown to function as a transcription factor which binds to the BAG1 promoter and represses BAG1 transcription. {ECO:0000269\|PubMed:22567091}.
Interaction	Q15834:CCDC85B; NbExp=2; IntAct=EBI-743375, EBI-739674; P62136:PPP1CA; NbExp=2; IntAct=EBI-743375, EBI-357253;
Similarity	Belongs to the MICOS complex subunit Mic19 family. Metazoan Mic19 subfamily. {ECO:0000305}.
Similarity	Contains 1 CHCH domain. {ECO:0000305}.
Subcellular Location	Mitochondrion inner membrane {ECO:0000250}; Lipid-anchor {ECO:0000250}; Intermembrane side {ECO:0000250}. Cytoplasm {ECO:0000269\|PubMed:22567091}. Nucleus {ECO:0000269\|PubMed:22567091}.
Subunit	Interacts with HSPA1A/HSPA1B and OPA1, preferentially with the soluble OPA1 form (By similarity). Component of the mitochondrial contact site and cristae organizing system (MICOS) complex, composed of at least MINOS1/MIC10, CHCHD3/MIC19, CHCHD6/MIC25, APOOL/MIC27 and IMMT/MIC60. The complex associates with mitochondrial outer membrane proteins SAMM50, MTX1 and MTX2, and with HSPA9. {ECO:0000250, ECO:0000269\|PubMed:21081504, ECO:0000269\|PubMed:22114354, ECO:0000269\|PubMed:22228767}.
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. {ECO:0000269\|PubMed:22567091}.