Constitutively active STAT6 predisposes toward a lymphoproliferative disorder

Mark H. Kaplan, Sarita Sehra, Hua Chen Chang, John T. O'Malley, Anubhav N. Mathur, Heather A. Bruns

Research output: Contribution to journalArticle

8 Scopus citations

Abstract

Signal transducer and activator of transcription 6 (STAT6) is critical for IL-4 and IL-13 responses, and necessary for the normal development of Th2 cells. We previously generated mice that express a constitutively active STAT6 (STAT6VT) under control of the CD2 locus control region, which directs expression to the T-cell compartment. We now describe that a small proportion of these mice (∼5%) develop a spontaneous lymphoproliferative disease (LPD) that results in dramatic splenomegaly. The cell populations observed in the LPD spleens can be divided into 2 categories, those that are composed of mixed lineage cells and those that are predominantly T cells with a phenotype similar to that in autoimmune lymphoproliferative syndrome (ALPS) patients. These data suggest that while active STAT6 is not a transforming factor, expression in T cells predisposes toward the development of lymphoproliferative disorders.

Original languageEnglish (US)
Pages (from-to)4367-4369
Number of pages3
JournalBlood
Volume110
Issue number13
DOIs
StatePublished - Dec 15 2007

    Fingerprint

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Kaplan, M. H., Sehra, S., Chang, H. C., O'Malley, J. T., Mathur, A. N., & Bruns, H. A. (2007). Constitutively active STAT6 predisposes toward a lymphoproliferative disorder. Blood, 110(13), 4367-4369. https://doi.org/10.1182/blood-2007-06-098244