Journal
CELLULAR AND MOLECULAR LIFE SCIENCES
Volume 66, Issue 7, Pages 1209-1222Publisher
SPRINGER BASEL AG
DOI: 10.1007/s00018-008-8580-6
Keywords
Fanconi anemia; DNA repair; helicase; genomic stability; G quadruplex; FANCJ; XPD; CHL1; CHLR1; DOG1; RTEL
Categories
Funding
- NIH
- National Institute on Aging and the Fanconi Anemia Research Fund
- NATIONAL INSTITUTE ON AGING [ZIAAG000753, ZIAAG000741] Funding Source: NIH RePORTER
Ask authors/readers for more resources
The FANCJ family of DNA helicases is emerging as an important group of proteins for the prevention of human disease, cancer, and chromosomal instability. FANCJ was identified by its association with breast cancer, and is implicated in Fanconi Anemia. Proteins with sequence similarity to FANCJ are important for maintenance of genomic stability. Mutations in genes encoding proteins related to FANCJ, designated ChlR1 in human and Chl1p in yeast, result in sister chromatid cohesion defects. Nematodes mutated in dog-1 show germline as well as somatic deletions in genes containing guanine-rich DNA. Rtel knockout mice are embryonic lethal, and embryonic stem cells show telomere loss and chromosomal instability. FANCJ also shares sequence similarity with human XPD and yeast RAD3 helicases required for nucleotide excision repair. The recently solved structure of XPD has provided new insight to the helicase core and accessory domains of sequence related Superfamily 2 helicases. The functions and roles of members of the FANCJ-like helicase family will be discussed.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available