BRIP1 (FANCJ) helicase functions in direct unwinding of DNA

Stable Identifier
Reaction [transition]
Gallus gallus
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BRIP1, a DNA helicase also known as FANCJ, provides evidence that the Fanconi anemia repair pathway may involve unwinding of DNA. BRIP1 interacts with BRCA1 through the two BRCT repeats at the C terminus of BRCA1. This interaction may contribute to the BRCA1 role in repair of double stranded breaks as well as execution of the G2/M checkpoint following DNA damage. However, unlike human BRIP1, chicken BRIP1 lacks the conserved Ser-X-X-Phe motif in its C-terminus that is thought to be crucial for BRCA1 interaction. The deleterious effects of a Walker A box K52R mutation indicates that ATPase activity is essential for chicken BRIP1 function. A BRIP1-FANCC double mutant has additive effects on sensitivity towards cisplastin, indicating their simultaneous involvement in DNA cross link repair pathways. Since BRIP1 mutant cells are proficient for ubiquitination of FANCD2 (unlike FANCC mutants), BRIP1 has a function that is independent of BRCA1 and FANCC, and downstream of FANCD2 activation.
Literature References
PubMed ID Title Journal Year
16116421 The BRIP1 helicase functions independently of BRCA1 in the Fanconi anemia pathway for DNA crosslink repair

Hiom, K, Vandenberg, CJ, Bridge, WL, Franklin, RJ

Nat Genet 2005
Catalyst Activity

DNA helicase activity of FANCJ (BRIP1), Fanconi anemia group J protein homolog [nucleoplasm]

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