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Research Article Free access | 10.1172/JCI117751

Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency.

K Høgåsen, J H Jansen, T E Mollnes, J Hovdenes, and M Harboe

Institute of Immunology and Rheumatology, National Hospital, University of Oslo, Norway.

Find articles by Høgåsen, K. in: PubMed | Google Scholar

Institute of Immunology and Rheumatology, National Hospital, University of Oslo, Norway.

Find articles by Jansen, J. in: PubMed | Google Scholar

Institute of Immunology and Rheumatology, National Hospital, University of Oslo, Norway.

Find articles by Mollnes, T. in: PubMed | Google Scholar

Institute of Immunology and Rheumatology, National Hospital, University of Oslo, Norway.

Find articles by Hovdenes, J. in: PubMed | Google Scholar

Institute of Immunology and Rheumatology, National Hospital, University of Oslo, Norway.

Find articles by Harboe, M. in: PubMed | Google Scholar

Published March 1, 1995 - More info

Published in Volume 95, Issue 3 on March 1, 1995
J Clin Invest. 1995;95(3):1054–1061. https://doi.org/10.1172/JCI117751.
© 1995 The American Society for Clinical Investigation
Published March 1, 1995 - Version history
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Abstract

We have recently described hereditary membranoproliferative glomerulonephritis type II in the pig. All affected animals had excessive complement activation, revealed as low plasma C3, elevated plasma terminal complement complex, and massive deposits of complement in the renal glomeruli, and eventually died of renal failure within 11 wk of birth. The aim of the present study was to investigate the cause of complement activation in this disease. Transfusion of normal porcine plasma to affected piglets inhibited complement activation and increased survival. Plasma was successively fractionated and the complement inhibitory effect of each fraction tested in vivo. A single chain 150-kD protein which showed the same complement inhibitory effect as whole plasma was finally isolated. Immunologic cross-reactivity, functional properties, and NH2-terminal sequence identified the protein as factor H. By Western blotting and enzyme immunoassay, membranoproliferative glomerulonephritis-affected piglets were demonstrated to be subtotally deficient in factor H. At 1 wk of age, median (range) factor H concentration was 1.6 mg/liter (1.1-2.3) in deficient animals (n = 13) and 51 mg/liter (26-98) in healthy littermates (n = 52). Our data show that hereditary porcine membrano-proliferative glomerulonephritis type II is caused by factor H deficiency.

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