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

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

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 (lin neg) 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 lin neg cells provide a tractable in vitro system for studies of myelodysplastic syndrome.

Original languageEnglish
Pages (from-to)1394-1397
Number of pages4
JournalHaematologica
Volume93
Issue number9
DOIs
Publication statusPublished - 2008 Sep 1
Externally publishedYes

Fingerprint

Myelodysplastic Syndromes
Apoptosis
Gene Fusion
Cell Death
nuclear pore complex protein 98
In Vitro Techniques

Keywords

  • Apoptosis
  • HOXD13
  • Mouse model
  • Myelodysplastic syndromes
  • NUP98

ASJC Scopus subject areas

  • Hematology

Cite this

Impaired differentiation and apoptosis of hematopoietic precursors in a mouse model of myelodysplastic syndrome. / Choi, Chul Won; Chung, Yang Jo; Slape, Christopher; Aplan, Peter D.

In: Haematologica, Vol. 93, No. 9, 01.09.2008, p. 1394-1397.

Research output: Contribution to journalArticle

Choi, Chul Won ; Chung, Yang Jo ; Slape, Christopher ; Aplan, Peter D. / Impaired differentiation and apoptosis of hematopoietic precursors in a mouse model of myelodysplastic syndrome. In: Haematologica. 2008 ; Vol. 93, No. 9. pp. 1394-1397.
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