Gene Symbol: Fancc
Description: Fanconi anemia, complementation group C
Alias: Facc, Fanconi anemia group C protein homolog
Species: mouse
Products:     Fancc

Top Publications

  1. Greenberg R, Allsopp R, Chin L, Morin G, DePinho R. Expression of mouse telomerase reverse transcriptase during development, differentiation and proliferation. Oncogene. 1998;16:1723-30 pubmed
    ..The widespread expression of mTERT in primary cells and mouse tissues could explain the increased frequency of spontaneous immortalization of mouse cells in culture and tumorigenesis in vivo. ..
  2. Kruyt F, Hoshino T, Liu J, Joseph P, Jaiswal A, Youssoufian H. Abnormal microsomal detoxification implicated in Fanconi anemia group C by interaction of the FAC protein with NADPH cytochrome P450 reductase. Blood. 1998;92:3050-6 pubmed
    ..We propose that FAC plays a fundamental role in vivo by attenuating the activity of RED, thereby regulating a major detoxification pathway in mammalian cells. ..
  3. Noll M, Battaile K, Bateman R, Lax T, Rathbun K, Reifsteck C, et al. Fanconi anemia group A and C double-mutant mice: functional evidence for a multi-protein Fanconi anemia complex. Exp Hematol. 2002;30:679-88 pubmed
    ..We generated mice with a targeted mutation in fanca and crossed them with fancc disrupted animals...
  4. Luebben S, Kawabata T, Akre M, Lee W, Johnson C, O Sullivan M, et al. Helq acts in parallel to Fancc to suppress replication-associated genome instability. Nucleic Acids Res. 2013;41:10283-97 pubmed publisher
    ..Here, we investigated this question in mice using the Helq(gt) and Fancc(-) strains...
  5. Chen M, Tomkins D, Auerbach W, McKerlie C, Youssoufian H, Liu L, et al. Inactivation of Fac in mice produces inducible chromosomal instability and reduced fertility reminiscent of Fanconi anaemia. Nat Genet. 1996;12:448-51 pubmed
    ..Homozygous male and female mice also had compromised gametogenesis, leading to markedly impaired fertility, a characteristic of FA patients. Thus, inactivation of Fac replicates some of the features of the human disease. ..
  6. Kamimae Lanning A, Goloviznina N, Kurre P. Fetal origins of hematopoietic failure in a murine model of Fanconi anemia. Blood. 2013;121:2008-12 pubmed publisher significant late gestational lethality and profound quantitative and qualitative deficiencies in the murine Fancc(-/-) fetal liver hematopoietic stem and progenitor cell pool...
  7. Nadler J, Braun R. Fanconi anemia complementation group C is required for proliferation of murine primordial germ cells. Genesis. 2000;27:117-23 pubmed
    ..Mice mutant for the Fanconi anemia complementation group C locus (Fancc) have reduced germ cell numbers as early as embryonic day E12...
  8. Zhang X, Sejas D, Qiu Y, Williams D, Pang Q. Inflammatory ROS promote and cooperate with the Fanconi anemia mutation for hematopoietic senescence. J Cell Sci. 2007;120:1572-83 pubmed
    ..using the genomic instability syndrome Fanconi anemia mouse model deficient for the complementation-group-C gene (Fancc)...
  9. Freie B, Li X, Ciccone S, Nawa K, Cooper S, Vogelweid C, et al. Fanconi anemia type C and p53 cooperate in apoptosis and tumorigenesis. Blood. 2003;102:4146-52 pubmed
    ..Here we show that ionizing radiation (IR) induces modestly elevated levels of p53 in cells from FA type C (Fancc) mutant mice and that inactivation of Trp53 rescues tumor necrosis factor alpha-induced apoptosis in myeloid cells ..

More Information


  1. Whitney M, Royle G, Low M, Kelly M, Axthelm M, Reifsteck C, et al. Germ cell defects and hematopoietic hypersensitivity to gamma-interferon in mice with a targeted disruption of the Fanconi anemia C gene. Blood. 1996;88:49-58 pubmed
    ..Progenitor cells from fac knock-out mice were hypersensitive to interferon gamma. This previously unrecognized phenotype may form the basis for BM failure in human FA. ..
  2. Huard C, Tremblay C, Helsper K, Delisle M, Schindler D, Levesque G, et al. Fanconi anemia proteins interact with CtBP1 and modulate the expression of the Wnt antagonist Dickkopf-1. Blood. 2013;121:1729-39 pubmed publisher
    ..Of the fifteen FA proteins, Fanconi anemia group C (FANCC) is one of eight FA core complex components of the FA pathway...
  3. Cumming R, Lightfoot J, Beard K, Youssoufian H, O Brien P, Buchwald M. Fanconi anemia group C protein prevents apoptosis in hematopoietic cells through redox regulation of GSTP1. Nat Med. 2001;7:814-20 pubmed
    The Fanconi anemia group C protein (FANCC) plays an important role in hematopoiesis by ensuring the survival of hematopoietic progenitor cells through an unknown mechanism...
  4. Hays L, Zodrow D, Yates J, Deffebach M, Jacoby D, Olson S, et al. Cigarette smoke induces genetic instability in airway epithelial cells by suppressing FANCD2 expression. Br J Cancer. 2008;98:1653-61 pubmed publisher
    ..Here, we show that cigarette smoke condensate (CSC) inhibited translation of FANCD2 mRNA (but not FANCC or FANCG) in normal airway epithelial cells and that this suppression of FANCD2 expression was sufficient to ..
  5. Fagerlie S, Koretsky T, Torok Storb B, Bagby G. Impaired type I IFN-induced Jak/STAT signaling in FA-C cells and abnormal CD4+ Th cell subsets in Fancc-/- mice. J Immunol. 2004;173:3863-70 pubmed
    The Fanconi anemia (FA) group C protein, FANCC, interacts with STAT1 following stimulation with IFN-gamma and is required for proper docking of STAT1 at the IFN-gamma receptor alpha-chain (IFN-gammaRalpha, IFN-gammaR1)...
  6. Haneline L, Broxmeyer H, Cooper S, Hangoc G, Carreau M, Buchwald M, et al. Multiple inhibitory cytokines induce deregulated progenitor growth and apoptosis in hematopoietic cells from Fac-/- mice. Blood. 1998;91:4092-8 pubmed
    ..Together these data suggest a role of Fac in affecting the signaling of multiple cytokine pathways and support cytokine-mediated apoptosis as a major mechanism responsible for BM failure observed in FA patients. ..
  7. Sejas D, Rani R, Qiu Y, Zhang X, Fagerlie S, Nakano H, et al. Inflammatory reactive oxygen species-mediated hemopoietic suppression in Fancc-deficient mice. J Immunol. 2007;178:5277-87 pubmed
    ..and innate immunity in FA hemopoietic cells using mice deficient in Fanconi complementation group C gene (Fancc)...
  8. Li X, Li L, Li J, Sipple J, Schick J, Mehta P, et al. Concomitant inactivation of foxo3a and fancc or fancd2 reveals a two-tier protection from oxidative stress-induced hydrocephalus. Antioxid Redox Signal. 2014;21:1675-92 pubmed publisher
    ..More than 50% of Foxo3a(-/-) Fancc(-/-) or Foxo3a(-/-) Fancd2(-/-) mice die during embryonic development or within 6 months of life as a result of ..
  9. Strathdee C, Duncan A, Buchwald M. Evidence for at least four Fanconi anaemia genes including FACC on chromosome 9. Nat Genet. 1992;1:196-8 pubmed
    ..3 through in situ hybridization. These results suggest that mutations in at least four different genes lead to FA, a degree of genetic heterogeneity comparable to that of other DNA repair disorders. ..
  10. Pulliam A, Hobson M, Ciccone S, Li Y, Chen S, Srour E, et al. AMD3100 synergizes with G-CSF to mobilize repopulating stem cells in Fanconi anemia knockout mice. Exp Hematol. 2008;36:1084-90 pubmed publisher
  11. Li X, Le Beau M, Ciccone S, Yang F, Freie B, Chen S, et al. 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. Blood. 2005;105:3465-71 pubmed
    ..Here we show that ex vivo culture of Fancc(-/-) bone marrow cells results in a time-dependent increase in apoptosis of primitive Fancc(-/-) progenitor cells ..
  12. Zhang X, Li J, Sejas D, Pang Q. Hypoxia-reoxygenation induces premature senescence in FA bone marrow hematopoietic cells. Blood. 2005;106:75-85 pubmed
    ..Here we show that expansion of Fancc-/- BM cells enriched for progenitor and stem cells was significantly decreased after 2 continuous cycles of ..
  13. Tisljar K, Deussing J, Peters C. Cathepsin J, a novel murine cysteine protease of the papain family with a placenta-restricted expression. FEBS Lett. 1999;459:299-304 pubmed
    ..Ctsj was mapped to mouse chromosome 13 in the vicinity of cathepsin L suggesting that cathepsin J may have arisen by gene duplication from cathepsin L or a common ancestral gene. ..
  14. Saadatzadeh M, Bijangi Vishehsaraei K, Kapur R, Haneline L. Distinct roles of stress-activated protein kinases in Fanconi anemia-type C-deficient hematopoiesis. Blood. 2009;113:2655-60 pubmed publisher
    ..Previously, enhanced apoptosis of Fanconi anemia type C-deficient (Fancc(-/-)) progenitors was shown to involve aberrant p38 MAPK activation...
  15. Garbati M, Hays L, Keeble W, Yates J, Rathbun R, Bagby G. FANCA and FANCC modulate TLR and p38 MAPK-dependent expression of IL-1? in macrophages. Blood. 2013;122:3197-205 pubmed publisher
    Hematopoietic stem and progenitor cells with inactivated Fanconi anemia (FA) genes, FANCA and FANCC, are hypersensitive to inflammatory cytokines...
  16. Luebben S, Kawabata T, Johnson C, O Sullivan M, Shima N. A concomitant loss of dormant origins and FANCC exacerbates genome instability by impairing DNA replication fork progression. Nucleic Acids Res. 2014;42:5605-15 pubmed publisher
    ..To understand this further, we introduced a null allele of Fancc (Fancc-), encoding a member of the FA core complex, into the Mcm4chaos3 background...
  17. Hu L, Huang W, Hjort E, Eklund E. Increased Fanconi C expression contributes to the emergency granulopoiesis response. J Clin Invest. 2013;123:3952-66 pubmed publisher
    ..that activates phagocyte effector genes during the innate immune response, activates the gene encoding Fanconi C (Fancc) in murine myeloid progenitor cells...
  18. Anur P, Yates J, Garbati M, Vanderwerf S, Keeble W, Rathbun K, et al. p38 MAPK inhibition suppresses the TLR-hypersensitive phenotype in FANCC- and FANCA-deficient mononuclear phagocytes. Blood. 2012;119:1992-2002 pubmed publisher
    Fanconi anemia, complementation group C (FANCC)-deficient hematopoietic stem and progenitor cells are hypersensitive to a variety of inhibitory cytokines, one of which, TNF?, can induce BM failure and clonal evolution in Fancc-deficient ..
  19. Wevrick R, Clarke C, Buchwald M. Cloning and analysis of the murine Fanconi anemia group C cDNA. Hum Mol Genet. 1993;2:655-62 pubmed
    ..We have now cloned the murine homologue of the human FACC cDNA. The mouse cDNA (Facc) shares 79% amino acid sequence similarity with the human gene product...
  20. Du W, Amarachintha S, Wilson A, Pang Q. SCO2 Mediates Oxidative Stress-Induced Glycolysis to Oxidative Phosphorylation Switch in Hematopoietic Stem Cells. Stem Cells. 2016;34:960-71 pubmed publisher
    ..These findings demonstrate p53-mediated OXPHOS function as a compensatory alteration in FA HSCs to ensure a functional but mildly impaired energy metabolism and suggest a cautious approach to manipulating p53 signaling in FA. ..
  21. Du W, Amarachintha S, Wilson A, Pang Q. Hyper-active non-homologous end joining selects for synthetic lethality resistant and pathological Fanconi anemia hematopoietic stem and progenitor cells. Sci Rep. 2016;6:22167 pubmed publisher
    ..Disruption of the FA core complex skews PARP1 function in DSB repair and led to hyper-active NHEJ in Fanca(-/-) or Fancc(-/-) HSPCs...
  22. Zhang T, Wilson A, Mahmood Ali A, Namekawa S, Andreassen P, Ruhikanta Meetei A, et al. Loss of Faap20 Causes Hematopoietic Stem and Progenitor Cell Depletion in Mice Under Genotoxic Stress. Stem Cells. 2015;33:2320-30 pubmed publisher lethally irradiated recipient mice, with milder phenotype as compared to HSPCs from Fanca(-) (/) (-) or Fancc(-) (/) (-) mice. Faap20(-) (/) (-) mice are susceptible to mitomycin C (MMC)-induced pancytopenia...
  23. Krasnoshtein F, Buchwald M. Developmental expression of the Fac gene correlates with congenital defects in Fanconi anemia patients. Hum Mol Genet. 1996;5:85-93 pubmed
    ..As well, expression in rapidly dividing progenitors is consistent with hypotheses regarding the nature of the basic defect in FA: a role of the protein in DNA repair or protection from oxygen toxicity. ..
  24. Navarro S, Meza N, Quintana Bustamante O, Casado J, Jacome A, McAllister K, et al. Hematopoietic dysfunction in a mouse model for Fanconi anemia group D1. Mol Ther. 2006;14:525-35 pubmed
    ..The hematopoietic phenotype associated with the Brca2(Delta27/Delta27) mutation suggests that this FA-D1 mouse model will constitute an important tool for the development of new therapies for FA, including gene therapy. ..
  25. Saadatzadeh M, Bijangi Vishehsaraei K, Hong P, Bergmann H, Haneline L. Oxidant hypersensitivity of Fanconi anemia type C-deficient cells is dependent on a redox-regulated apoptotic pathway. J Biol Chem. 2004;279:16805-12 pubmed the activation of a redox-dependent pathway may participate in the pro-apoptotic phenotype of primary Fancc -/- cells in response to oxidative stress...
  26. Pasteris N, Nagata K, Hall A, Gorski J. Isolation, characterization, and mapping of the mouse Fgd3 gene, a new Faciogenital Dysplasia (FGD1; Aarskog Syndrome) gene homologue. Gene. 2000;242:237-47 pubmed
    ..We conclude that Fgd3 is a new and novel member of the FGD1 family of RhoGEF proteins. ..
  27. Müller L, Milsom M, Harris C, Vyas R, Brumme K, Parmar K, et al. Overcoming reprogramming resistance of Fanconi anemia cells. Blood. 2012;119:5449-57 pubmed publisher
    ..These data define the role of the FA pathway in reprogramming and provide a strategy for future translational applications of patient-specific FA iPSCs. ..
  28. Liu Y, Ballman K, Li D, Khan S, Derr Yellin E, Shou W, et al. Impaired function of Fanconi anemia type C-deficient macrophages. J Leukoc Biol. 2012;91:333-40 pubmed publisher
    ..inflammation resolution, and antigen presentation for acquired immunity, we examined whether macrophages from Fancc-/- mice exhibit impaired function...
  29. Kato Y, Alavattam K, Sin H, Meetei A, Pang Q, Andreassen P, et al. FANCB is essential in the male germline and regulates H3K9 methylation on the sex chromosomes during meiosis. Hum Mol Genet. 2015;24:5234-49 pubmed publisher
    ..Taken together, these results indicate that FANCB functions at critical stages of germ cell development and reveal a novel function of the FA pathway in the regulation of H3K9 methylation in the germline. ..
  30. Abdul Sater Z, Cerabona D, Potchanant E, Sun Z, Enzor R, He Y, et al. FANCA safeguards interphase and mitosis during hematopoiesis in vivo. Exp Hematol. 2015;43:1031-1046.e12 pubmed publisher
    ..Our data indicate that FA/BRCA signaling functions as an in vivo gatekeeper of genomic integrity throughout interphase and mitosis, which may have implications for future targeted therapies in FA and FA-deficient cancers. ..
  31. Du W, Amarachintha S, Wilson A, Pang Q. The immune receptor Trem1 cooperates with diminished DNA damage response to induce preleukemic stem cell expansion. Leukemia. 2017;31:423-433 pubmed publisher
    ..Our study implicates diminishing DDR as a root cause of FA leukemogenesis, which subsequently collaborates with other signaling pathways for leukemogenic transformation. ..
  32. Skinner A, O Neill S, Grompe M, Kurre P. CXCR4 induction in hematopoietic progenitor cells from Fanca(-/-), -c(-/-), and -d2(-/-) mice. Exp Hematol. 2008;36:273-82 pubmed publisher
    ..Hypoxia conditioning of lineage-depleted Fancc(-/-) progenitors also reduced oxidative stress, improved in vitro migration and led to improved chimerism in ..
  33. Wevrick R, Barker J, Nadeau J, Szpirer C, Buchwald M. Mapping of the murine and rat Facc genes and assessment of flexed-tail as a candidate mouse homolog of Fanconi anemia group C. Mamm Genome. 1993;4:440-4 pubmed
    ..sensitivity of Fanconi anemia cells to DNA cross-linking agents, and we recently cloned its mouse homolog (Facc). In this report, we localized Facc to mouse Chromosome (Chr) 13 and its rat homolog to rat Chr 17...
  34. Naz R, Rajesh C. Gene knockouts that cause female infertility: search for novel contraceptive targets. Front Biosci. 2005;10:2447-59 pubmed
    ..These molecules will provide novel targets of contraception including contraceptive vaccine development. ..
  35. Ueda F, Sumi K, Tago K, Kasahara T, Funakoshi Tago M. Critical role of FANCC in JAK2 V617F mutant-induced resistance to DNA cross-linking drugs. Cell Signal. 2013;25:2115-24 pubmed publisher
    ..We first found that the expression of FANCC, a member of the Fanconi anemia (FA) proteins, was significantly induced by JAK2 V617F mutant through activation ..
  36. Otsuki T, Nagakura S, Wang J, Bloom M, Grompe M, Liu J. Tumor necrosis factor-alpha and CD95 ligation suppress erythropoiesis in Fanconi anemia C gene knockout mice. J Cell Physiol. 1999;179:79-86 pubmed
    ..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. ..
  37. Pulliam Leath A, Ciccone S, Nalepa G, Li X, Si Y, Miravalle L, et al. Genetic disruption of both Fancc and Fancg in mice recapitulates the hematopoietic manifestations of Fanconi anemia. Blood. 2010;116:2915-20 pubmed publisher
    ..Here we show that double-mutant Fancc(-/-);Fancg(-/-) mice develop spontaneous hematologic sequelae including bone marrow failure, AML, MDS and complex ..
  38. Rhee D, Wang Y, Mizesko M, Zhou F, Haneline L, Liu Y. FANCC suppresses short telomere-initiated telomere sister chromatid exchange. Hum Mol Genet. 2010;19:879-87 pubmed publisher
    ..b>FANCC is one of the most commonly mutated FA genes in FA patients and the FANCC subtype tends to have a relatively early ..
  39. Milsom M, Schiedlmeier B, Bailey J, Kim M, Li D, Jansen M, et al. Ectopic HOXB4 overcomes the inhibitory effect of tumor necrosis factor-{alpha} on Fanconi anemia hematopoietic stem and progenitor cells. Blood. 2009;113:5111-20 pubmed publisher
    ..Because HSCs derived from Fanconi anemia (FA) knockout mice are hypersensitive to TNF-alpha, we studied Fancc(-/-) HSCs to determine the physiologic effects of HOXB4 on TNF-alpha sensitivity and the relationship of these ..
  40. Li X, Yang Y, Yuan J, Hong P, Freie B, Orazi A, et al. Continuous in vivo infusion of interferon-gamma (IFN-gamma) preferentially reduces myeloid progenitor numbers and enhances engraftment of syngeneic wild-type cells in Fancc-/- mice. Blood. 2004;104:1204-9 pubmed
    ..Since myeloid progenitors from Fancc-/- mice and human Fanconi anemia group C protein (FANCC) patients have increased apoptosis in response to ..
  41. Sumpter R, Sirasanagandla S, Fernández A, Wei Y, Dong X, Franco L, et al. Fanconi Anemia Proteins Function in Mitophagy and Immunity. Cell. 2016;165:867-81 pubmed publisher
    ..Here, we describe an essential role for FA genes in two forms of selective autophagy. Genetic deletion of Fancc blocks the autophagic clearance of viruses (virophagy) and increases susceptibility to lethal viral encephalitis...
  42. Cerabona D, Sun Z, Nalepa G. Leukemia and chromosomal instability in aged Fancc-/- mice. Exp Hematol. 2016;44:352-7 pubmed publisher
    ..Here we describe that aging Fancc(-/-) mice are prone to genomically unstable AML and other hematologic neoplasms...
  43. Pang Q, Fagerlie S, Christianson T, Keeble W, Faulkner G, Diaz J, et al. The Fanconi anemia protein FANCC binds to and facilitates the activation of STAT1 by gamma interferon and hematopoietic growth factors. Mol Cell Biol. 2000;20:4724-35 pubmed
    ..STAT1 activation is suppressed in IFN-stimulated FA cells, an abnormality corrected by transduction of normal FANCC cDNA...
  44. Li J, Sejas D, Zhang X, Qiu Y, Nattamai K, Rani R, et al. TNF-alpha induces leukemic clonal evolution ex vivo in Fanconi anemia group C murine stem cells. J Clin Invest. 2007;117:3283-95 pubmed
    ..Hematopoietic stem cells bearing inactivating mutations of FA complementation group C (FANCC) are genetically unstable and hypersensitive to apoptotic cytokine cues including IFN-gamma and TNF-alpha, but ..
  45. Freie B, Ciccone S, Li X, Plett P, Orschell C, Srour E, et al. A role for the Fanconi anemia C protein in maintaining the DNA damage-induced G2 checkpoint. J Biol Chem. 2004;279:50986-93 pubmed
    ..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...
  46. Zhou T, Hasty P, Walter C, Bishop A, Scott L, Rebel V. Myelodysplastic syndrome: an inability to appropriately respond to damaged DNA?. Exp Hematol. 2013;41:665-74 pubmed publisher
    ..Moreover, in view of our current understanding of how DNA damage response and repair pathways are affected by age in hematopoietic stem cells, we also explore how this might relate to MDS development. ..
  47. Hays L, Keeble W, Yates J, Rathbun R, Koretsky T, Olson S, et al. Human FANCC is hypomorphic in murine Fancc-deficient cells. Blood. 2010;116:2057-60 pubmed publisher
    b>Fancc suppresses cross-linker-induced genotoxicity, modulates growth-inhibitory cytokine responses, and modulates endotoxin responses...
  48. Alavattam K, Kato Y, Sin H, Maezawa S, Kowalski I, Zhang F, et al. Elucidation of the Fanconi Anemia Protein Network in Meiosis and Its Function in the Regulation of Histone Modifications. Cell Rep. 2016;17:1141-1157 pubmed publisher
    ..We reveal a network of FA and DDR proteins in which FA core factors FANCA, FANCB, and FANCC are essential for FANCD2 foci formation, whereas BRCA1 (FANCS), MDC1, and RNF8 are required for BRCA2 (FANCD1) ..
  49. Servadio A, McCall A, Zoghbi H, Eicher E. Mapping of the Sca1 and pcd genes on mouse chromosome 13 provides evidence that they are different genes. Genomics. 1995;29:812-3 pubmed
  50. Sertorio M, Amarachintha S, Wilson A, Pang Q. Loss of Fancc Impairs Antibody-Secreting Cell Differentiation in Mice through Deregulating the Wnt Signaling Pathway. J Immunol. 2016;196:2986-94 pubmed publisher
    ..In this study, we investigated the effect of FA deficiency in B cell function using the Fancc mouse model...
  51. Zhang Q, Marquez Loza L, Eaton L, Duncan A, Goldman D, Anur P, et al. Fancd2-/- mice have hematopoietic defects that can be partially corrected by resveratrol. Blood. 2010;116:5140-8 pubmed publisher
    ..We conclude that Fancd2(-/-) mice have readily quantifiable hematopoietic defects, and that this model is well suited for pharmacologic screening studies. ..
  52. Battaile K, Bateman R, Mortimer D, Mulcahy J, Rathbun R, Bagby G, et al. In vivo selection of wild-type hematopoietic stem cells in a murine model of Fanconi anemia. Blood. 1999;94:2151-8 pubmed
    ..No systemic side effects were observed. Our results show that in vivo selection for wild-type hematopoietic stem cells occurs in FA and that it is enhanced by MMC administration. ..
  53. Hadjur S, Ung K, Wadsworth L, Dimmick J, Rajcan Separovic E, Scott R, et al. Defective hematopoiesis and hepatic steatosis in mice with combined deficiencies of the genes encoding Fancc and Cu/Zn superoxide dismutase. Blood. 2001;98:1003-11 pubmed
    ..deficiencies of the cytosolic Cu/Zn superoxide dismutase (Sod1) and Fanconi anemia complementation group C (Fancc) genes...
  54. Cooke H, Saunders P. Mouse models of male infertility. Nat Rev Genet. 2002;3:790-801 pubmed
    ..Targeted mutagenesis in the mouse provides a powerful method to analyse these steps and has provided new insights into the origins of male infertility. ..
  55. Si Y, Ciccone S, Yang F, Yuan J, Zeng D, Chen S, et al. Continuous in vivo infusion of interferon-gamma (IFN-gamma) enhances engraftment of syngeneic wild-type cells in Fanca-/- and Fancg-/- mice. Blood. 2006;108:4283-7 pubmed
    ..Hypersensitivity of Fancc-/- cells to interferon-gamma (IFN-gamma), a nongenotoxic immune-regulatory cytokine, enhances engraftment of ..
  56. Vanderwerf S, Svahn J, Olson S, Rathbun R, Harrington C, Yates J, et al. TLR8-dependent TNF-(alpha) overexpression in Fanconi anemia group C cells. Blood. 2009;114:5290-8 pubmed publisher
    ..anemia (FA) cells and contributes to the hematopoietic defects seen in FA complementation group C-deficient (Fancc(-/-)) mice...
  57. Li X, Plett P, Yang Y, Hong P, Freie B, Srour E, et al. Fanconi anemia type C-deficient hematopoietic stem/progenitor cells exhibit aberrant cell cycle control. Blood. 2003;102:2081-4 pubmed
    ..Here, we used Fanconi anemia complementation type C-deficient (Fancc-/-) mice to demonstrate that Fancc-/- phenotypically defined cell populations enriched for hematopoietic stem and ..
  58. Larin M, Gallo D, Tamblyn L, Yang J, Liao H, Sabat N, et al. Fanconi anemia signaling and Mus81 cooperate to safeguard development and crosslink repair. Nucleic Acids Res. 2014;42:9807-20 pubmed publisher
    ..Using mice deficient in both Mus81 and the FA pathway protein FancC, we show both proteins cooperate in parallel pathways, as concomitant loss of FancC and Mus81 triggered cell-type-..
  59. Hahn H, Christiansen J, Wicking C, Zaphiropoulos P, Chidambaram A, Gerrard B, et al. A mammalian patched homolog is expressed in target tissues of sonic hedgehog and maps to a region associated with developmental abnormalities. J Biol Chem. 1996;271:12125-8 pubmed
    ..Human PATCHED maps to human chromosome 9q22.3, the candidate region for the nevoid basal cell carcinoma syndrome. Patched expression is compatible with the congenital defects observed in the nevoid basal cell carcinoma syndrome. ..
  60. Si Y, Pulliam A, Linka Y, Ciccone S, Leurs C, Yuan J, et al. Overnight transduction with foamyviral vectors restores the long-term repopulating activity of Fancc-/- stem cells. Blood. 2008;112:4458-65 pubmed publisher
    ..2- to 4 day ex vivo manipulation of bone marrow from mice containing a disruption of the homologue of human FANCC (Fancc) results in a time-dependent increase in apoptosis and a risk for malignant transformation of hematopoietic ..