Centromere protein X (CENPX)

Centromere protein X (CENPX) is a highly conserved DNA-binding protein that plays dual roles in chromosome biology: (1) it is essential for proper kinetochore architecture and function, supporting accurate chromosome alignment and segregation during mitosis; (2) it acts as a DNA repair factor within the Fanconi anemia (FA) pathway, partnering with CENP-S (MHF1) and FANCM to facilitate the resolution of complex DNA structures and support the repair of interstrand crosslinks and stalled replication forks. Its functions are critical for maintaining genome stability, and mutations in CENPX are associated with disorders such as Fanconi anemia and certain types of osteogenesis imperfecta

Other names

CENPXFAAP10MHF2STRA13CENP-XMGC14480Stimulated by retinoic acid gene 13 protein homologFanconi anemia-associated histone fold protein 2Retinoic acid-inducible gene D9 protein homologFanconi anemia-associated polypeptide of 10 kDa

Mechanisms primarily relate to modulation of the Fanconi anemia DNA repair pathway, particularly the monoubiquitination of the FANCI-FANCD2 complex in response to DNA damage. Drugs that induce DNA cross-links may interact functionally by challenging the repair capacities mediated by CENPX/FANCM/MHF complex.