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 language | English |
|---|---|
| Pages (from-to) | 4367-4369 |
| Number of pages | 3 |
| Journal | Blood |
| Volume | 110 |
| Issue number | 13 |
| DOIs | |
| State | Published - Dec 15 2007 |
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ASJC Scopus subject areas
- Hematology
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Constitutively active STAT6 predisposes toward a lymphoproliferative disorder. / Kaplan, Mark; Sehra, Sarita; Chang, Hua Chen; O'Malley, John T.; Mathur, Anubhav N.; Bruns, Heather A.
In: Blood, Vol. 110, No. 13, 15.12.2007, p. 4367-4369.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Constitutively active STAT6 predisposes toward a lymphoproliferative disorder
AU - Kaplan, Mark
AU - Sehra, Sarita
AU - Chang, Hua Chen
AU - O'Malley, John T.
AU - Mathur, Anubhav N.
AU - Bruns, Heather A.
PY - 2007/12/15
Y1 - 2007/12/15
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=39649113142&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=39649113142&partnerID=8YFLogxK
U2 - 10.1182/blood-2007-06-098244
DO - 10.1182/blood-2007-06-098244
M3 - Article
C2 - 17878403
AN - SCOPUS:39649113142
VL - 110
SP - 4367
EP - 4369
JO - Blood
JF - Blood
SN - 0006-4971
IS - 13
ER -