| Modification Notes |
CHCHD4 (coiled-coil-helix-coiled-coil-helix domain containing 4), also known as MIA40, is an 142 amino acid protein that contains one CHCH domain and localizes to the mitochondrial intermembrane space. Expressed in a variety of tissues with particularly high expression in kidney and liver, CHCHD4 exists as a homooligomer that is required for the folding of small cysteine-containing proteins. Specifically, CHCHD4 is thought to function via a disulfide relay system that oxidizes precursor proteins, thereby promoting their folding within mitochondria. CHCHD4 is expressed as multiple alternatively spliced isoforms that are encoded by a gene which maps to human chromosome 3. Chromosome 3 is made up of about 214 million bases encoding over 1,100 genes, including a chemokine receptor (CKR) gene cluster and a variety of human cancer-related gene loci. |