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JCI early table of contents for May 24, 2013

ion of an immune system response mechanism known as complement. Normally the complement system helps to clear pathogens from the body. It consists of a number of small proteins that, upon immune activation, are broken up into fragments that bind to foreign material or pathogen-infected cells to trigger their destruction. In C3 glomerulopathies, the complement fragments accumulate in the kidney, causing damage. In this issue of the Journal of Clinical Investigation, Santiago Rodrguez de Crdoba and colleagues at the Centro de Investigaciones Biolgicas in Madrid, Spain identifed mutations in complement regulating protein, factor H, which increased complement activation. These studies help to define how factor H functions in complement regulation and provide a greater understanding of C3 glomerulopathy pathology. As Michael Holers of the University of Colorado explains in the accompanying commentary, variants in factor H genes are linked to multiple autoimmune and inflammatory diseases. Studies like this one may help to direct the development of new therapies.

C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation

Santiago Rodrguez de Crdoba
Centro de Investigaciones Biolgicas (CSIC), Madrid, UNK, ESP
Phone: 34 91 8373112; E-mail:

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Human C3 glomerulopathy provides unique insights into complement factor Hrelated protein function

Michael Holers
University of Colorado Denver School of Medicine, Denver, CO, USA
Phone: 303-724-7609; E-mail:<

Contact: Jillian Hurst
Journal of Clinical Investigation

Page: 1 2 3 4 5 6 7 8

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