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Proc Natl Acad Sci U S A. 1998 Oct 27;95(22):13085-90.

The fanconi anemia pathway requires FAA phosphorylation and FAA/FAC nuclear accumulation.

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  • 1Institute of Medical Science, University of Tokyo, Tokyo 108, Japan.

Abstract

Fanconi anemia (FA) is an autosomal recessive cancer susceptibility syndrome with at least eight complementation groups (A-H). Two FA genes, corresponding to complementation groups A and C, have been cloned, but the function of the FAA and FAC proteins remains unknown. We have recently shown that the FAA and FAC proteins bind and form a nuclear complex. In the current study, we analyzed the FAA and FAC proteins in normal lymphoblasts and lymphoblasts from multiple FA complementation groups. In contrast to normal controls, FA cells derived from groups A, B, C, E, F, G, and H were defective in the formation of the FAA/FAC protein complex, the phosphorylation of the FAA protein, and the accumulation of the FAA/FAC protein complex in the nucleus. These biochemical events seem to define a signaling pathway required for the maintenance of genomic stability and normal hematopoiesis. Our results support the idea that multiple gene products cooperate in the FA Pathway.

PMID:
9789045
PMCID:
PMC23717
[PubMed - indexed for MEDLINE]
Free PMC Article
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