Mutation of E2F2 in mice causes enhanced T lymphocyte proliferation, leading to the development of autoimmunity.

E2Fs are important regulators of proliferation, differentiation, and apoptosis. Here we characterize the phenotype of mice deficient in E2F2. We show that E2F2 is required for immunologic self-tolerance. E2F2(-/-) mice develop late-onset autoimmune features, characterized by widespread inflammatory...

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Detalles Bibliográficos
Autores: Murga, Matilde, Fernandez-Capetillo, Oscar, Field, S J, Moreno, B, Borlado, L R, Fujiwara, Y, Balomenos, D, Vicario, A, Carrera, A C, Orkin, S H, Greenberg, M E, Zubiaga, A M
Tipo de recurso: artículo
Fecha de publicación:2001
País:España
Institución:Instituto de Salud Carlos III (ISCIII)
Repositorio:Repisalud
Idioma:inglés
OAI Identifier:oai:repisalud.isciii.es:20.500.12105/17701
Acceso en línea:http://hdl.handle.net/20.500.12105/17701
Access Level:acceso abierto
Palabra clave:Cell Cycle Proteins
DNA-Binding Proteins
Animals
Apoptosis
Autoimmune Diseases
Autoimmunity
Cell Division
Chimera
Clonal Deletion
E2F Transcription Factors
E2F1 Transcription Factor
E2F2 Transcription Factor
Gene Expression Regulation
Glomerulonephritis, Membranoproliferative
H-Y Antigen
Humans
Immunologic Memory
Inflammation
Jurkat Cells
Lymphocyte Activation
Male
Mice
Mice, Inbred BALB C
Mice, Inbred C57BL
Mice, Knockout
Mutagenesis, Site-Directed
Receptors, Antigen, T-Cell
Recombinant Fusion Proteins
Repressor Proteins
S Phase
Self Tolerance
Splenomegaly
T-Lymphocytes
Thymus Gland
Transcription Factors
Transfection
Descripción
Sumario:E2Fs are important regulators of proliferation, differentiation, and apoptosis. Here we characterize the phenotype of mice deficient in E2F2. We show that E2F2 is required for immunologic self-tolerance. E2F2(-/-) mice develop late-onset autoimmune features, characterized by widespread inflammatory infiltrates, glomerular immunocomplex deposition, and anti-nuclear antibodies. E2F2-deficient T lymphocytes exhibit enhanced TCR-stimulated proliferation and a lower activation threshold, leading to the accumulation of a population of autoreactive effector/memory T lymphocytes, which appear to be responsible for causing autoimmunity in E2F2-deficient mice. Finally, we provide support for a model to explain E2F2's unexpected role as a suppressor of T lymphocyte proliferation. Rather than functioning as a transcriptional activator, E2F2 appears to function as a transcriptional repressor of genes required for normal S phase entry, particularly E2F1.