DISEASES

Disease-gene associations mined from literature

Literature on CENPS

CENPS [ENSP00000308583]

Centromere protein S; DNA-binding component of the Fanconi anemia (FA) core complex. Required for the normal activation of the FA pathway, leading to monoubiquitination of the FANCI-FANCD2 complex in response to DNA damage, cellular resistance to DNA cross-linking drugs, and prevention of chromosomal breakage . In complex with CENPX (MHF heterodimer), crucial cofactor for FANCM in both binding and ATP-dependent remodeling of DNA. Stabilizes FANCM . In complex with CENPX and FANCM (but not other FANC proteins), rapidly recruited to blocked forks and promotes gene conversion at blocked replication forks . In complex with CENPT, CENPW and CENPX (CENP-T-W-S-X heterotetramer), involved in the formation of a functional kinetochore outer plate, which is essential for kinetochore-microtubule attachment and faithful mitotic progression . As a component of MHF and CENP-T-W-S-X complexes, binds DNA and bends it to form a nucleosome-like structure . DNA- binding function is fulfilled in the presence of CENPX, with the following preference for DNA substates: Holliday junction > double- stranded > splay arm > single-stranded. Does not bind DNA on its own . ECO:0000269|PubMed:20347428, ECO:0000269|PubMed:20347429,

Synonyms:  CENPS,  A0A0U1RQL5,  CENPS-CORT,  K7ESB0,  Q8N2Z9 ...

Linkouts:  STRING  Pharos  UniProt