Published online 4 July 2008
Haematologica, Vol 93, Issue 9, 1394-1397 doi:10.3324/haematol.13042
Copyright © 2008 by Ferrata Storti Foundation
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Myelodysplastic Syndromes

Impaired differentiation and apoptosis of hematopoietic precursors in a mouse model of myelodysplastic syndrome

Chul Won Choi, Yang Jo Chung, Christopher Slape, Peter D. Aplan

Genetics Branch, Center for Cancer Research, National Cancer Institute, National Institute of Health, Bethesda, MD, USA

Correspondence: Peter D. Aplan, Navy 8, Room 5101 8901 Wisconsin Ave, Bethesda MD 20889 USA.E-mail:aplanp{at}mail.nih.gov

Expression of a NUP98-HOXD13 (NHD13) fusion gene, initially identified in a patient with myelodysplastic syndrome, leads to a highly penetrant myelodysplastic syndrome in mice that recapitulates all of the key features of the human disease. Expansion of undifferentiated lineage negative (linneg) hematopoietic precursors that express NHD13 was markedly inhibited (30-fold) in vitro. Decreased expansion was accompanied by decreased production of terminally differentiated cells, indicating impaired differentiation of NHD13 precursors. Rather than differentiate, the majority (80%) of NHD13 linneg precursors underwent apoptotic cell death when induced to differentiate. These findings demonstrate that NHD13 linneg cells provide a tractable in vitro system for studies of myelodysplastic syndrome.

Key words: myelodysplastic syndromes, mouse model, NUP98, HOXD13, apoptosis.




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M. Fontenay and E. Gyan
Apoptotic pathways to death in myelodysplastic syndromes
Haematologica, September 1, 2008; 93(9): 1288 - 1292.
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