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FANCA

Function

DNA repair protein that may operate in a postreplication repair or a cell cycle checkpoint function. May be involved in interstrand DNA cross-link repair and in the maintenance of normal chromosome stability.

Involvement in disease

Fanconi anemia, complementation group A

FANCA

A disorder affecting all bone marrow elements and resulting in anemia, leukopenia and thrombopenia. It is associated with cardiac, renal and limb malformations, dermal pigmentary changes, and a predisposition to the development of malignancies. At the cellular level it is associated with hypersensitivity to DNA-damaging agents, chromosomal instability (increased chromosome breakage) and defective DNA repair.

None

The disease is caused by variants affecting the gene represented in this entry.

Post-translational modifications

Phosphorylation is required for the formation of the nuclear complex. Not phosphorylated in cells derived from groups A, B, C, E, F, G, and H.

Cellular localization

Alternative names

FAA, FACA, FANCH, FANCA, Fanconi anemia group A protein, Protein FACA

swissprot:O15360 omim:607139 entrezGene:2175