title: C3b receptor (CR1) on phagocytic cells from SLE patients: analysis of the defect and familial study.
abstract: In vitro CR1-dependent phagocytosis of C3b-coated erythrocytes, by monocytes and PMN, was found to be significantly decreased in SLE patients. This was in many cases related to a specific defect of CR1 receptors, since the FcR-ingestion of EIgG was normal. On the other hand, CR1 levels of PMN stimulated by FMLP were also found to be decreased in SLE patients, while both the expression of circulating PMN (cells isolated at 4 degrees C) and the total cellular CR1 content were normal. On the basis of the available data, we propose that the impaired phagocytosis is due to a functional defect of CR1 or a defective anchorage of the receptor to the plasma membrane, possibly related to the decreased capacity of CR1 to be up-regulated by FMLP. To study the importance of the genetic background in the CR1 abnormalities, the families of 22 young SLE patients, in which the onset of the disease had occurred before the age of 15, were studied. The expression of CR1 on erythrocytes, and the total CR1 content of PMN, in parents and siblings of these patients did not differ significantly from normal controls. By contrast, the ingestion of EIgGC3b by PMN from healthy relatives of these patients was decreased (65% of the normal mean of PI), while EIgG phagocytosis was normal. A relation between this CR1 functional defect and the familial occurrence of autoimmune disorders is therefore possible.
authors.affiliation: CNRS UA.122-INSERM U.25, Hôpital Necker, Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Mir
authors.forename: F
authors.initials: F
authors.lastname: Porteu
authors.forename: M
authors.initials: M
authors.lastname: Levy
authors.forename: P
authors.initials: P
authors.lastname: Lesavre
authors.forename: L
authors.initials: L
authors.lastname: Halbwachs-Mecarelli
mesh_terms: D004912:Erythrocytes / Q000276:immunology; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000235:genetics / Q000276:immunology*; D009000:Monocytes / Q000276:immunology; D009504:Neutrophils / Q000276:immunology; D010586:Phagocytes / Q000276:immunology*; D010587:Phagocytosis; D011951:Receptors, Complement / Q000032:analysis*; D017463:Receptors, Complement 3b
chemical_list: D011951:Receptors, Complement; D017463:Receptors, Complement 3b