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Fanconi anemia group A protein; protein FACA (FANCA FAA FACA FANCH)

Function: - DNA repair protein - postreplication repair - cell cycle checkpoint function - interstrand DNA cross-link repair - maintenance of normal chromosome stability - phosphorylated upon DNA damage, probably by ATM or ATR - phosphorylation is required for the formation of the nuclear complex - not phosphorylated in cells derived from groups A, B, C, E, F, G, & H - anti-oncoprotein ? - component of multisubunit FA complex (not found in FA patients) Compartment: - nucleus, cytoplasm - major form is nuclear - minor form is cytoplasmic Alternative splicing: named isoforms=2 Pathology: - defects in FANCA are a cause of Fanconi anemia (FA)

Related

Fanconi anemia

General

nuclear protein phosphoprotein protein subunit

Properties

SIZE: entity length = 1455 aa MW = 163 kD COMPARTMENT: cytoplasm cell nucleus MOTIF: nuclear translocation signal {18-34} Ser phosphorylation site {S849} Ser phosphorylation site {S850} Ser phosphorylation site {S858} Ser phosphorylation site {S1449}

Database Correlations

OMIM correlations MORBIDMAP 607139 UniProt O15360 Pfam PF03511 Entrez Gene 2175 Kegg hsa:2175

References

  1. UniProt :accession O15360
  2. Atlas of Genetics & Cytogenetics in Oncology & Haematology http://atlasgeneticsoncology.org/genes/FA1ID102.html
  3. Fanconi Anemia mutation Database http://www.rockefeller.edu/fanconi/mutate/jumpa.html
  4. GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/FANCA
  5. NIEHS-SNPs http://egp.gs.washington.edu/data/fanca/

Component-of

Fanconi anemia complementation group complex (FA complex)