Fanconi anemia type C-deficient hematopoietic stem/progenitor cells exhibit aberrant cell cycle control

Xiaxin Li, P. Artur Plett, Yanzhu Yang, Ping Hong, Brian Freie, Edward F. Srour, Christie M. Orschell, D. Wade Clapp, Laura S. Haneline

Research output: Contribution to journalArticle

25 Scopus citations


The pathogenesis of bone marrow failure in Fanconi anemia is poorly understood. Suggested mechanisms include enhanced apoptosis secondary to DNA damage and altered inhibitory cytokine signaling. Recent data determined that disrupted cell cycle control of hematopoietic stem and/or progenitor cells disrupts normal hematopoiesis with increased hematopoietic stem cell cycling resulting in diminished function and increased sensitivity to cell cycle-specific apoptotic stimuli. Here, we used Fanconi anemia complementation type C-deficient (Fancc-/-) mice to demonstrate that Fancc -/- phenotypically defined cell populations enriched for hematopoietic stem and progenitor cells exhibit increased cycling. In addition, we established that the defect in cell cycle regulation is not a compensatory mechanism from enhanced apoptosis occurring in vivo. Collectively, these data provide a previously unrecognized phenotype in Fancc-/- hematopoietic stem/progenitor cells, which may contribute to the progressive bone marrow failure in Fanconi anemia.

Original languageEnglish (US)
Pages (from-to)2081-2084
Number of pages4
Issue number6
StatePublished - Sep 15 2003


ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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