CHCHD1 (Coiled-Coil-Helix-Coiled-Coil-Helix Domain Containing 1) is a Protein Coding gene. The CHCHD1 gene, located on 10q22.2, is conserved in chimpanzee, Rhesus monkey, dog, cow, mouse, rat, chicken, zebrafish, and frog. CHCHD1 is a kind of mitochondrial ribosomal proteins (MRPs), which play essential roles in synthesizing the essential subunits of oxidative phosphorylation (OXPHOS) complexes and can cause various diseases in humans. Researchers have demonstrated the essential roles of CHCHD1 in mitochondrial protein synthesis by siRNA knock-down studies, which had significant effects on the expression of mitochondrially encoded proteins. The gene is commonly expressed in the adrenal, colon, and other tissues. Among its related pathways are Organelle biogenesis and maintenance and Mitochondrial translation.