Coiled-coil-helix-coiled-coil-helix domain-containing protein 6 (CHCHD6)

Coiled-coil-helix-coiled-coil-helix domain-containing protein 6 (CHCHD6) is a mitochondrial protein predominantly localized to the inner mitochondrial membrane as a core component of the MICOS (mitochondrial contact site and cristae organizing system) complex[1][3][5]. It is essential for the maintenance of cristae morphology and mitochondrial inner membrane architecture, directly interacting with other MICOS proteins like mitofilin and CHCHD3[1][3]. Disruption or loss of CHCHD6 impairs mitochondrial structure, leads to bioenergetic failure, and increases susceptibility to genotoxic stress, thereby reducing cell growth and ATP production[1][2]. In Alzheimer’s disease models and human tissues, CHCHD6 is selectively reduced, linking it to amyloid processing, mitochondrial dysfunction, and neurodegeneration, suggesting a potential therapeutic target for AD and possibly cancer[1][2][5]. It does not belong to classical drug target categories like receptors or enzymes but is considered a target due to its role in disease-related cellular processes.

Other names

MICOS complex subunit MIC25CHCM1MIC25MGC13016PPP1R23Mic25MICOS25coiled-coil-helix cristae morphology protein 1coiled-coil-helix-coiled-coil-helix domain-containing protein 6protein phosphatase 1 regulatory subunit 23coiled-coil-helix cristae morphology 1mitochondrial contact site and cristae organizing system subunit 25