What question did this study set out to answer?

To determine how subtotal complement factor H deficiency influences susceptibility to C3 glomerulopathy and atypical hemolytic uremic syndrome in vivo.

September 15, 2015Journal of the American Society of NephrologyOpen Access

Partial Complement Factor H Deficiency Associates with C3 Glomerulopathy and Thrombotic Microangiopathy

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Authors

KVKatherine A. VernonBroad Institute MRMarieta M. RusevaAge UK HCH. Terence CookImperial College Healthcare NHS Trust

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Implication

Animal study reveals that partial complement factor H deficiency triggers kidney disease in mice, highlighting dual pathways to glomerulopathy and microangiopathy.

Key Points

To determine how subtotal complement factor H deficiency influences susceptibility to C3 glomerulopathy and atypical hemolytic uremic syndrome in vivo.
Generated a mouse model with hepatocyte-specific complement factor H deficiency to achieve reduced plasma factor H levels.
Evaluated spontaneous renal pathology and induced complement activation within the kidney using accelerated serum nephrotoxic nephritis.
Mice with hepatocyte-specific factor H deficiency exhibited secondary plasma C3 reduction and mesangial C3 accumulation consistent with C3 glomerulopathy without plasma C5 depletion.
Animals did not develop spontaneous thrombotic microangiopathy, but developed severe, C5-dependent thrombotic microangiopathy following the induction of nephrotoxic nephritis.

Cite This Study

Vernon et al. (2015) studied this question.

synapsesocial.com/papers/6a754f151078cf7de2b2098a https://doi.org/10.1681/asn.2015030295

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Implication

Animal study reveals that partial complement factor H deficiency triggers kidney disease in mice, highlighting dual pathways to glomerulopathy and microangiopathy.

Partial Complement Factor H Deficiency Associates with C3 Glomerulopathy and Thrombotic Microangiopathy

Discussion

Member takes

Implication

Key Points

Cite This Study

Partial Complement Factor H Deficiency Associates with C3 Glomerulopathy and Thrombotic Microangiopathy

Discussion

Member takes

Implication

Key Points

Cite This Study

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