Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation

Martínez-Barricarte, Rubén; Heurich, Meike; Valdés-Cañedo, Francisco; Vázquez‐Martul, Eduardo; Torreira, Eva; Montes, Tamara; Tortajada, Agustín; Pinto, Sheila; López‐Trascasa, Margarita; Paul Morgan, B.; Llorca, Óscar; L. Harris, Claire; Rodrı́guez de Córdoba, Santiago

doi:10.1172/jci43343

Public

Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation

Published in Journal of Clinical Investigation • Sep 17, 2010

Authors:

Rubén Martínez-Barricarte

Meike Heurich

Francisco Valdés-Cañedo

Abstract

Dense deposit disease (DDD) is a severe renal disease characterized by accumulation of electron-dense material in the mesangium and glomerular basement membrane. Previously, DDD has been associated with deficiency of factor H (fH), a plasma regulator of the alternative pathway (AP) of complement act...

Subject

C3-convertase

Complement system

Alternative complement pathway

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