Coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial (CHCHD10)

Coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial (CHCHD10) is a mitochondrial inner membrane protein highly enriched at cristae junctions, encoded by the CHCHD10 gene on chromosome 22. CHCHD10 is a component of the MICOS complex, a key regulator of mitochondrial inner membrane morphology and cristae formation. The protein’s structure features a nonstructured N-terminal region, a hydrophobic helix, and a C-terminal CHCH domain with two disulfide bonds. Functional studies indicate that CHCHD10 is involved in maintaining cristae morphology, mitochondrial respiration, and can influence apoptosis and mitochondrial genome stability. Mutations in CHCHD10 are linked to a spectrum of neurodegenerative disorders, most notably ALS and frontotemporal dementia, through disruption of mitochondrial dynamics, respiratory function, and cellular stress responses.

Other names

CHCHD10Coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrialProtein N27C7-4C22orf16MIX17AFTDALS2IMMDSMAJMIX17 homolog A