title: Haploinsufficiency of Bcl11b suppresses the progression of ATM-deficient T cell lymphomas.
abstract: Bcl11b is a transcription factor important for T cell development and also a tumor-suppressor gene that is hemizygously inactivated in ~10% human T cell acute lymphoblastic leukemia (T-ALL) and several murine T-ALL models, including ATM(-/-) thymic lymphomas. Here we report that heterozygous loss of Bcl11b (Bcl11b(+/-)) unexpectedly reduced lethal thymic lymphoma in ATM(-/-) mice by suppressing lymphoma progression, but not initiation. The suppression was associated with a T cell-mediated immune response in ATM(-/-)Bcl11b(+/-) mice, revealing a haploid insufficient function of Bcl11b in immune modulation against lymphoma and offering an explanation for the complex relationship between Bcl11b status with T-ALL prognosis.
authors.affiliation: Department of Pediatrics, Division of Hematology, Oncology and Stem Cell Transplantation, Columbia University, 1130 St Nicholas Ave, New York, NY, 10032, USA. kpinkney@mhs.net.|Current address: Joe DiMaggio's Children's Hospital, 1150 North 35th Avenue, Suite 100, Hollywood, FL, 33021, USA. kpinkney@mhs.net.
authors.forename: Kerice A
authors.initials: KA
authors.lastname: Pinkney
authors.affiliation: Institute for Cancer Genetics, Columbia University, 1130 St Nicholas Ave, RM 503B, New York, NY, 10032, USA. jw759@columbia.edu.|Herbert Irving Comprehensive Cancer Research Center, Columbia University, 1130 St Nicholas Ave, New York, NY, 10032, USA. jw759@columbia.edu.
authors.forename: Wenxia
authors.initials: W
authors.lastname: Jiang
authors.affiliation: Institute for Cancer Genetics, Columbia University, 1130 St Nicholas Ave, RM 503B, New York, NY, 10032, USA. bl2530@columbia.edu.
authors.forename: Brian J
authors.initials: BJ
authors.lastname: Lee
authors.affiliation: Institute for Cancer Genetics, Columbia University, 1130 St Nicholas Ave, RM 503B, New York, NY, 10032, USA. dl2970@columbia.edu.
authors.forename: Denis G
authors.initials: DG
authors.lastname: Loredan
authors.affiliation: Institute for Cancer Genetics, Columbia University, 1130 St Nicholas Ave, RM 503B, New York, NY, 10032, USA. cl3103@columbia.edu.
authors.forename: Chen
authors.initials: C
authors.lastname: Li
authors.affiliation: Herbert Irving Comprehensive Cancer Research Center, Columbia University, 1130 St Nicholas Ave, New York, NY, 10032, USA. gb96@columbia.edu.|Department of Pathology and Cell Biology, College for Physicians and Surgeons, Columbia University, 1130 St Nicholas Ave, New York, NY, 10032, USA. gb96@columbia.edu.
authors.forename: Govind
authors.initials: G
authors.lastname: Bhagat
authors.affiliation: Institute for Cancer Genetics, Columbia University, 1130 St Nicholas Ave, RM 503B, New York, NY, 10032, USA. sz2296@columbia.edu.|Herbert Irving Comprehensive Cancer Research Center, Columbia University, 1130 St Nicholas Ave, New York, NY, 10032, USA. sz2296@columbia.edu.|Department of Pediatrics, Division of Hematology, Oncology and Stem Cell Transplantation, Columbia University, 1130 St Nicholas Ave, New York, NY, 10032, USA. sz2296@columbia.edu.|Department of Pathology and Cell Biology, College for Physicians and Surgeons, Columbia University, 1130 St Nicholas Ave, New York, NY, 10032, USA. sz2296@columbia.edu.
authors.forename: Shan
authors.initials: S
authors.lastname: Zha
mesh_terms: D000818:Animals; D018450:Disease Progression; D005786:Gene Expression Regulation; D057895:Haploinsufficiency / Q000276:immunology*; D006801:Humans; D016399:Lymphoma, T-Cell / Q000276:immunology* / Q000473:pathology; D051379:Mice; D012097:Repressor Proteins / Q000378:metabolism*; D025521:Tumor Suppressor Proteins / Q000378:metabolism*
chemical_list: D012097:Repressor Proteins; D025521:Tumor Suppressor Proteins