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Gene Review

Fancc  -  Fanconi anemia, complementation group C

Mus musculus

Synonyms: BB116513, Fac, Facc, Fanconi anemia group C protein homolog, Protein FACC
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Disease relevance of Fancc

  • Here we show that the Fanconi anemia complementation group C protein (Fancc) is necessary for proper function of the DNA damage-induced G2/M checkpoint in vitro and in vivo [1].
  • Fancc(-/-) HSCs transduced with a retrovirus encoding rFancc exhibited a repopulating ability that approached wild-type levels [2].
  • Interestingly, approximately 30% of primary recipients (7 of 22) transplanted with uncorrected Fancc(-/-) cells developed a range of hematopoietic abnormalities including pancytopenia and BM hypoplasia similar to individuals with FA [2].
  • Conversely, inhibition of PKR function by enforced expression of a dominant-negative inhibitory mutant of PKR (PKRDelta6) substantially reduced the IFN and dsRNA hypersensitivity of FANCC(-/-) cells [3].
  • Developmental expression of the Fac gene correlates with congenital defects in Fanconi anemia patients [4].

High impact information on Fancc


Chemical compound and disease context of Fancc


Biological context of Fancc

  • These results suggested that the altered redox state likely present in Fancc(-/-) Sod1(-/-) hematopoietic progenitors was responsible for an impairment of cell proliferation or survival [9].
  • Mice mutant for the Fanconi anemia complementation group C locus (Fancc) have reduced germ cell numbers as early as embryonic day E12.5, suggesting the Fancc protein functions prior to meiosis in both sexes [10].
  • Here, we tested whether alterations in the activation of a redox-dependent pathway may participate in the pro-apoptotic phenotype of primary Fancc -/- cells in response to oxidative stress [11].
  • The mouse cDNA (Facc) shares 79% amino acid sequence similarity with the human gene product [12].
  • In this report, we localized Facc to mouse Chromosome (Chr) 13 and its rat homolog to rat Chr 17 [13].

Anatomical context of Fancc

  • The second phenotypic feature of Fancc(-/-) Sod1(-/-) mice was that of bone marrow hypocellularity accompanied by significant decreases in peripheral blood erythrocyte and leukocyte numbers as compared with wild-type controls [9].
  • This study demonstrates Fancc is required for mitotic proliferation of primordial germ cells [10].
  • Supporting our hypothesis that nitric oxide (NO) production might be a common effector in this sensitivity, we found that cytokine-mediated growth inhibition of Fancc(-/-) bone marrow cells was prevented by inhibiting NO synthase activity [14].
  • Retroviral-mediated expression of recombinant Fancc enhances the repopulating ability of Fancc-/- hematopoietic stem cells and decreases the risk of clonal evolution [2].
  • Fancc(-/-) mice injected with granulocyte colony-stimulating factor, erythropoietin, or both cytokines showed a delay in mitomycin C (MMC)-induced bone marrow (BM) failure compared to untreated mice [15].

Associations of Fancc with chemical compounds


Regulatory relationships of Fancc


Other interactions of Fancc

  • The characteristics of the Fancg(-/-) mice closely resemble those reported for Fancc and Fanca null mice, supporting a tight interdependence of the corresponding gene products in a common pathway [17].
  • In contrast, genetic disruption of both Fancc and Atm did not cooperate in the G2 checkpoint [1].
  • Impaired type I IFN-induced Jak/STAT signaling in FA-C cells and abnormal CD4+ Th cell subsets in Fancc-/- mice [18].
  • We conclude that mutation in the Fac protein induces heightened sensitivity to TNF-alpha and Fas receptor ligation, results that may explain the mechanism of anemia in FA-C patients [19].
  • Consequently, loss of a functional FANCC results in decreased activation of STAT1 following IFN-gamma stimulation [18].

Analytical, diagnostic and therapeutic context of Fancc


  1. A role for the Fanconi anemia C protein in maintaining the DNA damage-induced G2 checkpoint. Freie, B.W., Ciccone, S.L., Li, X., Plett, P.A., Orschell, C.M., Srour, E.F., Hanenberg, H., Schindler, D., Lee, S.H., Clapp, D.W. J. Biol. Chem. (2004) [Pubmed]
  2. Retroviral-mediated expression of recombinant Fancc enhances the repopulating ability of Fancc-/- hematopoietic stem cells and decreases the risk of clonal evolution. Haneline, L.S., Li, X., Ciccone, S.L., Hong, P., Yang, Y., Broxmeyer, H.E., Lee, S.H., Orazi, A., Srour, E.F., Clapp, D.W. Blood (2003) [Pubmed]
  3. Role of double-stranded RNA-dependent protein kinase in mediating hypersensitivity of Fanconi anemia complementation group C cells to interferon gamma, tumor necrosis factor-alpha, and double-stranded RNA. Pang, Q., Keeble, W., Diaz, J., Christianson, T.A., Fagerlie, S., Rathbun, K., Faulkner, G.R., O'Dwyer, M., Bagby, G.C. Blood (2001) [Pubmed]
  4. Developmental expression of the Fac gene correlates with congenital defects in Fanconi anemia patients. Krasnoshtein, F., Buchwald, M. Hum. Mol. Genet. (1996) [Pubmed]
  5. Inactivation of Fac in mice produces inducible chromosomal instability and reduced fertility reminiscent of Fanconi anaemia. Chen, M., Tomkins, D.J., Auerbach, W., McKerlie, C., Youssoufian, H., Liu, L., Gan, O., Carreau, M., Auerbach, A., Groves, T., Guidos, C.J., Freedman, M.H., Cross, J., Percy, D.H., Dick, J.E., Joyner, A.L., Buchwald, M. Nat. Genet. (1996) [Pubmed]
  6. Ex vivo culture of Fancc-/- stem/progenitor cells predisposes cells to undergo apoptosis, and surviving stem/progenitor cells display cytogenetic abnormalities and an increased risk of malignancy. Li, X., Le Beau, M.M., Ciccone, S., Yang, F.C., Freie, B., Chen, S., Yuan, J., Hong, P., Orazi, A., Haneline, L.S., Clapp, D.W. Blood (2005) [Pubmed]
  7. Fanconi anemia type C and p53 cooperate in apoptosis and tumorigenesis. Freie, B., Li, X., Ciccone, S.L., Nawa, K., Cooper, S., Vogelweid, C., Schantz, L., Haneline, L.S., Orazi, A., Broxmeyer, H.E., Lee, S.H., Clapp, D.W. Blood (2003) [Pubmed]
  8. Development and characterization of immortalized fibroblastoid cell lines from an FA(C) mouse model. Tomkins, D.J., Care, M., Carreau, M., Buchwald, M. Mutat. Res. (1998) [Pubmed]
  9. Defective hematopoiesis and hepatic steatosis in mice with combined deficiencies of the genes encoding Fancc and Cu/Zn superoxide dismutase. Hadjur, S., Ung, K., Wadsworth, L., Dimmick, J., Rajcan-Separovic, E., Scott, R.W., Buchwald, M., Jirik, F.R. Blood (2001) [Pubmed]
  10. Fanconi anemia complementation group C is required for proliferation of murine primordial germ cells. Nadler, J.J., Braun, R.E. Genesis (2000) [Pubmed]
  11. Oxidant hypersensitivity of Fanconi anemia type C-deficient cells is dependent on a redox-regulated apoptotic pathway. Saadatzadeh, M.R., Bijangi-Vishehsaraei, K., Hong, P., Bergmann, H., Haneline, L.S. J. Biol. Chem. (2004) [Pubmed]
  12. Cloning and analysis of the murine Fanconi anemia group C cDNA. Wevrick, R., Clarke, C.A., Buchwald, M. Hum. Mol. Genet. (1993) [Pubmed]
  13. Mapping of the murine and rat Facc genes and assessment of flexed-tail as a candidate mouse homolog of Fanconi anemia group C. Wevrick, R., Barker, J.E., Nadeau, J.H., Szpirer, C., Buchwald, M. Mamm. Genome (1993) [Pubmed]
  14. Increased sensitivity of Fancc-deficient hematopoietic cells to nitric oxide and evidence that this species mediates growth inhibition by cytokines. Hadjur, S., Jirik, F.R. Blood (2003) [Pubmed]
  15. Short-term granulocyte colony-stimulating factor and erythropoietin treatment enhances hematopoiesis and survival in the mitomycin C-conditioned Fancc(-/-) mouse model, while long-term treatment is ineffective. Carreau, M., Liu, L., Gan, O.I., Hitzler, J.K., Dick, J.E., Buchwald, M. Blood (2002) [Pubmed]
  16. The ATM/p53/p21 pathway influences cell fate decision between apoptosis and senescence in reoxygenated hematopoietic progenitor cells. Zhang, X., Li, J., Sejas, D.P., Pang, Q. J. Biol. Chem. (2005) [Pubmed]
  17. Reduced fertility and hypersensitivity to mitomycin C characterize Fancg/Xrcc9 null mice. Koomen, M., Cheng, N.C., van de Vrugt, H.J., Godthelp, B.C., van der Valk, M.A., Oostra, A.B., Zdzienicka, M.Z., Joenje, H., Arwert, F. Hum. Mol. Genet. (2002) [Pubmed]
  18. Impaired type I IFN-induced Jak/STAT signaling in FA-C cells and abnormal CD4+ Th cell subsets in Fancc-/- mice. Fagerlie, S.R., Koretsky, T., Torok-Storb, B., Bagby, G.C. J. Immunol. (2004) [Pubmed]
  19. Tumor necrosis factor-alpha and CD95 ligation suppress erythropoiesis in Fanconi anemia C gene knockout mice. Otsuki, T., Nagakura, S., Wang, J., Bloom, M., Grompe, M., Liu, J.M. J. Cell. Physiol. (1999) [Pubmed]
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