Fanconi anemia group B protein (FANCB)

Fanconi anemia group B protein (FANCB) is a core component of the Fanconi anemia (FA) DNA repair pathway, involved in repairing DNA interstrand crosslinks during DNA replication[2][3]. FANCB forms a central part of the FA core complex, assembling with FAAP100 and FANCL to create the catalytic E3 ubiquitin ligase subcomplex critical for the monoubiquitination of FANCD2 and FANCI[1][2]. This modification is essential for recruiting downstream nucleases and repair proteins to damaged DNA. Mutations in FANCB cause a severe, X-linked form of Fanconi anemia, leading to congenital abnormalities, bone marrow failure, cancer predisposition, and infertility, particularly in males[2]. FANCB does not appear to be a direct target for any approved drugs, but its essential role in DNA repair means its dysfunction markedly affects cell survival in response to DNA crosslinking agents[3].

Other names

Protein FACBFAAP95FAAP90FABFLJ34064Fanconi anemia-associated polypeptide of 95 kDaFanconi anemia complementation group B

As part of the FA core complex, FANCB enables E3 ubiquitin ligase activity, critical for monoubiquitinating FANCD2 and FANCI, which is a key step in DNA interstrand crosslink repair[1][2][3]