Rabbit anti-Human CHCHD3 Polyclonal Antibody

Uniprot Id

Q9NX63

Target Species

Human

Target Name

CHCHD3

Target Full Name

MICOS complex subunit MIC19

Target 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. Plays an important role in the maintenance of the MICOS complex stability and the mitochondrial cristae morphology.

Target Subcellular Location

Mitochondrion inner membrane; Lipid-anchor; Intermembrane side. Cytoplasm. Nucleus. Mitochondrion.

Target Protein Families

MICOS complex subunit Mic19 family, Metazoan Mic19 subfamily

Target 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.

Target Research Area

Tags & Cell Markers

Target Synonyms

CHCH3_HUMAN; CHCHD 3; Chchd3; Coiled coil helix coiled coil helix domain containing 3; Coiled coil helix coiled coil helix domain containing protein 3 mitochondrial; Coiled-coil-helix-coiled-coil-helix domain-containing protein 3; FLJ20420; MINOS3; mitochondrial; Mitochondrial inner membrane organizing system 3; PPP1R22; Protein phosphatase 1 regulatory subunit 22

Target Background

The protein encoded by this gene is an inner mitochondrial membrane scaffold protein. Absence of the encoded protein affects the structural integrity of mitochondrial cristae and leads to reductions in ATP production, cell growth, and oxygen consumption. This protein is part of the mitochondrial contact site and cristae organizing system (MICOS). Several transcript variants encoding different isoforms have been found for this gene.