Kidney Disease MPGN II/DDD/C3G

Membranoproliferative Glomerulonephritis (MPGN) is a rare kidney disorder which most frequently affects young patients. This disease which is currently also termed C3 glomerulopathy represents a spectrum of related disorders. The major diagnosis is based on the immunohistological evaluation of a kidney biopsy.The involvement of complement and autoimmune parameters such as the C3 nephritic factor, autoantibodies to Factor B, Factor H and to the C3 convertase  have frequently been reported. In addition, genetic mutations including the genes Factor H, C3 and the CFHR genes, i.e. CFHR2, CFHR5 and CFHR3 are reported in patients with MPGN /C3G. The Department of Infection Biology is interested in understanding the exact role of complement in the pathomechanisms of this severe kidney disorder. Our central aim is to understand the scenarios that result in the formation of autoimmune forms. In addition, we aim at defining how the various genetic alterations result in pathology and the heterogeneity of this disease. To allow a better understanding of the heterogeneity and pathophysiology we have established a registry that currently includes more than 250 patients suffering from the initial diagnosis MPGN I, or MPGNII. This allows us to define subtypes of the heterogeneous disease. Individual auto antibodies and mutant proteins are characterized to show how they affect and disrupt immune homeostasis. Using this approach and in close collaboration with clinical and medical doctors, new forms of diagnosis and therapy are being developed with regard to these severe kidney diseases.

Defective C3 convertase activity results in C3 glomerulopathy. C3 glomerulopathy cab be caused by gene mutations or by autoantibodies that bind to various epitopes of the C3 convertase.

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C3 Glomerulopathy - Immunsuppressive therapy for a 8 year old female patient with autoimmune C3 glomerulopathy reduced autoantibody titers and plasma levels of the complement activation product Ba. (Chen Q et al. 2012, New Engl J Med)

Leader

Prof. Dr. Peter F. Zipfel

Phone: +49 3641 532-1301 Email: peter.zipfel@leibniz-hki.de

Members

Steffi Hälbich

Phone: +49 3641 532-1175 Email: steffi.haelbich@leibniz-hki.de

Andrea Hartmann

Phone: +49 3641 532-1177 Email: andrea.hartmann@leibniz-hki.de

Ina Löschmann

Phone: +49 3641 532-1173 Email: ina.loeschmann@leibniz-hki.de

Ramona Rudnick

Phone: +49 3641 532-1166 Email: ramona.rudnick@leibniz-hki.de

Prof. Dr. Christine Skerka

Phone: +49 3641 532-1164 Email: christine.skerka@leibniz-hki.de

Monika von der Heide

Phone: +49 3641 532-1169 Email: Monika.vonderHeide@leibniz-hki.de

Publications

Currently there are no publications available in this context.