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Human Molecular Genetics, 2000, Vol. 9, No. 12 1805-1811
© 2000 Oxford University Press

Mice with a targeted disruption of the Fanconi anemia homolog Fanca

Ngan Ching Cheng1,2, Henri J. van de Vrugt1, Martin A. van der Valk3, Anneke B. Oostra1, Paul Krimpenfort2, Yne de Vries1, Hans Joenje1, Anton Berns2 and Fré Arwert,1,+

1Department of Clinical Genetics and Human Genetics, Free University Medical Center, Van der Boechorststraat 7, 1081 BT Amsterdam, The Netherlands, 2Division of Molecular Genetics and Center of Biomedical Genetics, The Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The Netherlands and 3Department of Experimental Animal Pathology, The Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The Netherlands

Fanconi anemia (FA) is a hereditary chromosomal instability syndrome with cancer predisposition. Bone marrow failure resulting in pancytopenia is the main cause of death of FA patients. Diagnosis of FA is based on their cellular hypersensitivity to DNA crosslinking agents and chromosome breakages. Somatic complementation experiments suggest the involvement of at least eight genes in FA. The gene for complementation group A (FANCA) is defective in the majority of FA patients. We show here that mice deficient of Fanca are viable and have no detectable developmental abnormalities. The hematological parameters showed a slightly decreased platelet count and a slightly increased erythrocyte mean cell volume in mice at young age, but this did not progress to anemia. Consistent with the clinical phenotype of FA patients, both male and female mice showed hypogonadism and impaired fertility. Furthermore, embryonic fibroblasts of the knock-out mice exhibited spontaneous chromosomal instability and were hyper-responsive to the clastogenic effect of the crosslinker mitomycin C.

+ To whom correspondence should be addressed. Tel: +31 20 4448270; Fax: +31 20 4448285; Email: f.arwert.humgen@med.vu.nl


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