Humanized C3 Mouse: A Novel Accelerated Model of C3 Glomerulopathy.
Kishor Devalaraja-NarashimhaKaroline MeagherYifan LuoCong HuangTheodore KaplanAnantharaman MuthuswamyGabor HalaszSarah CasanovaJohn O'BrienRebecca Peyser BoiarskyJohn McWhirterHans GartnerYu BaiScott MacDonnellChien LiuYing HuAdrianna LatuszekYi WeiSrinivasa PrasadTammy HuangGeorge YancopoulosAndrew MurphyWilliam OlsonBrian ZambrowiczLynn E MacdonaldLori G MortonPublished in: Journal of the American Society of Nephrology : JASN (2020)
The C3hu/hu mice are a useful model for C3G because they share many pathologic features consistent with the human disease. The C3G phenotype in C3hu/hu mice may originate from a dysregulated interaction of human C3 protein with multiple mouse complement proteins, leading to unregulated C3 activation via AP. The accelerated disease course in C3hu/hu mice may further enable preclinical studies to assess and validate new therapeutics for C3G.