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Hematopoietic Stem Cell Defects in Mice with Deficiency of Fancd2 or Usp1

4 May 2010

Abstract excerpt

Fanconi anemia (FA) is a human genetic disease characterized by a DNA repair defect and progressive bone marrow failure. Central events in the FA pathway are the monoubiquitination of the Fancd2 protein and the removal of ubiquitin by the deubiquitinating enzyme, Usp1. Here, we have investigated the role of Fancd2 and Usp1 in the maintenance and function of murine hematopoietic stem cells (HSCs). Bone marrow from...

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PMID: 20506303
DOI: 10.1002/stem.437

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