Complement factor H–related hybrid protein deregulates complement in dense deposit disease

Complement factor H-related hybrid protein deregulates complement in dense deposit disease.

The renal disorder C3 glomerulopathy with dense deposit disease (C3G-DDD) pattern results from complement dysfunction and primarily affects children and young adults. There is no effective treatment, and patients often progress to end-stage renal failure. A small fraction of C3G-DDD cases linked to factor H or C3 gene mutations as well as autoantibodies have been reported. Here, we examined an ...

Blockade of Alternative Complement Pathway in Dense Deposit Disease

A patient aged 17 with dense deposit disease associated with complement activation, circulating C3 Nef, and Factor H mutation presented with nephrotic syndrome and hypertension. Steroid therapy, plasma exchange, and rituximab failed to improve proteinuria and hypertension despite a normalization of the circulating sC5b9 complex. Eculizumab, a monoclonal antibody directed against C5, was used to...

Anti-Complement Autoantibodies in Membranoproliferative Glomerulonephritis and Dense Deposit Disease

The complement system is an essential part of innate immunity by its role in protection against infections, but it is also involved in waste disposal and in modulating the adaptive immune response. Under physiological conditions complement activation is effectively regulated to restrain it to the required targets and extent, and to prevent collateral host tissue damage. An imbalance between com...

Allelic Variants of Complement Genes Associated with Dense Deposit Disease

The alternative pathway of the complement cascade plays a role in the pathogenesis of dense deposit disease (DDD). Deficiency of complement factor H and mutations in CFH associate with the development of DDD, but it is unknown whether allelic variants in other complement genes also associate with this disease. We studied patients with DDD and identified previously unreported sequence alteration...

Indirect Complement Fixation in Foot-and-Mouth Disease