Welcome the Family of FANCJ-like Helicases to the Block of Genome Stability Maintenance Proteins |
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Authors: | Y Wu A N Suhasini R M Brosh Jr |
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Institution: | (1) Laboratory of Molecular Gerontology, National Institute on Aging, National Institutes of Health, NIH Biomedical Research Center, 251 Bayview Blvd, Baltimore, MD 21224, USA |
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Abstract: | 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.
Received 17 September 2008; received after revision 24 October 2008; accepted 28 October 2008 |
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Keywords: | " target="_blank"> Fanconi anemia DNA repair helicase genomic stability G quadruplex FANCJ XPD CHL1 CHLR1 DOG1 RTEL |
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