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Overexpression of the antiapoptotic gene Bfl-1 in B cells from patients with familial systemic lupus erythematosus

Service d'immuno-hématologie pédiatrique, Hôpital Debrousse, Hospices Civils de Lyon, Lyon, France

R Cimaz

Département de Pédiatrie, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France; Université Lyon 1, Lyon, France

B Ranchin

Département de Pédiatrie, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France

C Galambrun

Y Bertrand

Service d'immuno-hématologie pédiatrique, Hôpital Debrousse, Hospices Civils de Lyon, Lyon, France

R Bouvier

Laboratoire d'anatomopathologie, Hôpital Edouard Herriot, Hospices Civils De Lyon, France

F Rieux-Laucat

Inserm U768, Hôpital Necker Enfants Malades, Paris, France

M-C Trescol-Biemont

Inserm, U503, Lyon, France; Université de Lyon, Lyon, France; Université Lyon 1, Lyon, France; IFR 128, Lyon, France

P Cochat

Département de Pédiatrie, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France; Université Lyon 1, Lyon, France

N Bonnefoy-Berard

Inserm, U503, Lyon, France; Université de Lyon, Lyon, France; Université Lyon 1, Lyon, France; IFR 128, Lyon, France; bonnefoy{at}cervi-lyon.inserm.fr

Genetic determinants taking part in the development of systemic lupus erythematosus (SLE) are complex and not fully characterized. Dysregulated expression of genes involved in the control of apoptosis has been previously suggested. We report here a consanguineous family with SLE manifestations in three siblings associated in one of them with severe lymphoproliferative features. Laboratory studies showed no defect in CD95-mediated cell death. Screening expression of Bcl-2 family genes that regulate mitochondrial apoptosis pathway showed an overexpression of the antiapoptotic Bfl-1 gene. Real time RT-PCR analysis indicated that overexpression of Bfl-1 was restricted to B-cells, with normal expression in T-cells. Those results suggest that overexpression of Bfl-1 could result in impaired B-lymphocyte homeostasis and inappropriate immune response leading to autoimmune manifestations.

Key Words: apoptosis • B-cells • Bfl-1 • Bcl-2 • lupus

Lupus, Vol. 16, No. 2, 95-100 (2007)
DOI: 10.1177/0961203306075382


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