PMID: 11918676Mar 29, 2002Paper

Characterization, expression and complex formation of the murine Fanconi anaemia gene product Fancg

Genes to Cells : Devoted to Molecular & Cellular Mechanisms
H J van de VrugtF Arwert

Abstract

Fanconi anaemia (FA) is an autosomal recessive chromosomal instability disorder. Six distinct FA disease genes have been identified, the products of which function in an integrated pathway that is thought to support a nuclear caretaker function. Comparison of FA gene characteristics in different species may help to unravel the molecular function of the FA pathway. We have cloned the murine homologue of the Fanconi anaemia complementation group G gene, FANCG/XRCC9. The murine Fancg protein shows an 83% similarity to the human protein sequence, and has a predicted molecular weight of 68.5 kDa. Expression of mouse Fancg in human FA-G lymphoblasts fully corrects their cross-linker hypersensitivity. At mRNA and protein levels we detected the co-expression of Fancg and Fanca in murine tissues. In addition, mouse Fancg and Fanca proteins co-purify by immunoprecipitation. Upon transfection into Fanca-deficient mouse embryonic fibroblasts EGFP-Fancg chimeric protein was detectable in the nucleus. We identified a murine cDNA, Fancg, which cross-complements the cellular defect of human FA-G cells and thus represents a true homologue of human FANCG. Spleen, thymus and testis showed the highest Fancg expression levels. Although Fancg and Fa...Continue Reading

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Citations

Nov 26, 2009·Cancer Research·Henri J van de VrugtMarkus Grompe
Jun 2, 2012·Blood·Kim-Hien T DaoGrover C Bagby
Jun 18, 2003·BMC Blood Disorders·Michel AubéMadeleine Carreau
Mar 3, 2007·Breast Cancer Research and Treatment·Petra van der GroepPaul J van Diest
Jun 23, 2007·Genes to Cells : Devoted to Molecular & Cellular Mechanisms·Stacie StoneMaureen E Hoatlin
Aug 21, 2004·Blood·Sonia FrancoMaría A Blasco

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