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Coiled-coil-helix-coiled-coil-helix domain-containing protein 7 (CHCHD7) is a protein encoded by the CHCHD7 gene in humans. CHCHD7 is part of the twin CX9C protein family and is characterized by a distinctive coiled-coil-helix-coiled-coil-helix (CHCH) motif stabilized by two disulfide bonds[2][3][5]. The protein is localized to the mitochondrial intermembrane space where it is involved in the assembly of mitochondrial respiratory chain complexes, particularly cytochrome c oxidase (COX) analogous to its yeast homolog COX23[6][8]. CHCHD7 is thought to serve as a substrate for the Mia40-dependent import machinery, a system responsible for the import and folding of small cysteine-containing proteins within the mitochondrial intermembrane space[2][3]. Disease associations include certain carcinomas, but its precise molecular mechanisms in disease pathology, therapeutic potential as a target, and druggability remain poorly characterized at this time[8][10].
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