Damage control and its costs: BM failure in Fanconi anemia stems from overactive p53/p21
- PMID: 22770237
- PMCID: PMC3402214
- DOI: 10.1016/j.stem.2012.06.013
Damage control and its costs: BM failure in Fanconi anemia stems from overactive p53/p21
Abstract
Despite having well-characterized disease-associated mutations, the mechanisms underlying the progressive bone marrow failure and cancer susceptibility of Fanconi anemia have been unclear. In this issue of Cell Stem Cell, Ceccaldi et al. identify an overactive p53/p21 stress response and cell cycle arrest as an underlying cause that starts during fetal development.
Copyright © 2012 Elsevier Inc. All rights reserved.
References
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- Ceccaldi R, Parmar K, Mouly E, Delord M, Kim JM, Regairaz M, Pla M, Vasquez N, Zhang QS, Pondarre C, et al. Bone marrow failure in Fanconi anemia is triggered by an exacerbated p53/p21 DNA damage response that impairs hematopoietic stem and progenitor cells. Cell Stem Cell. 2012 this issue. - PMC - PubMed
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- Fanconi G. Familiäre infantile perniziosaartige Anämie (perniziöses Blutbild und Konstitution) Jahrbuch für Kinderheilkunde und physische Erziehung (Wien) 1927;117:257–280. (in German)
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- Ceccaldi R, Briot D, Larghero J, Vasquez N, Dubois d'Enghien C, Chamousset D, Noguera ME, Waisfisz Q, Hermine O, Pondarre C, et al. Spontaneous abrogation of the G2 DNA damage checkpoint has clinical benefits but promotes leukemogenesis in Fanconi anemia patients. J Clin Invest. 2011;121:184–194. - PMC - PubMed
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