作者
Kishor Devalaraja-Narashimha, Karoline Meagher, Yifan Luo, Cong Huang, Theodore Kaplan, Anantharaman Muthuswamy, Gabor Halasz, Sarah Casanova, John O’Brien, Rebecca Peyser Boiarsky, John McWhirter, Hans Gartner, Yu Bai, Scott MacDonnell, Chien Liu, Ying Hu, Adrianna Latuszek, Yi Wei, Srinivasa Prasad, Tammy Huang, George Yancopoulos, Andrew Murphy, William Olson, Brian Zambrowicz, Lynn Macdonald, Lori G Morton
发表日期
2021/1/1
期刊
Journal of the American Society of Nephrology
卷号
32
期号
1
页码范围
99-114
出版商
LWW
简介
Background
C3 glomerulopathy (C3G) is characterized by the alternative-pathway (AP) hyperactivation induced by nephritic factors or complement gene mutations. Mice deficient in complement factor H (CFH) are a classic C3G model, with kidney disease that requires several months to progress to renal failure. Novel C3G models can further contribute to understanding the mechanism behind this disease and developing therapeutic approaches.
Methods
A novel, rapidly progressing, severe, murine model of C3G was developed by replacing the mouse C3 gene with the human C3 homolog using VelociGene technology. Functional, histologic, molecular, and pharmacologic assays characterize the presentation of renal disease and enable useful pharmacologic interventions in the humanized C3 (C3 hu/hu) mice.
Results
The C3 hu/hu mice exhibit increased morbidity early in life and die by about 5–6 months of age …
学术搜索中的文章
K Devalaraja-Narashimha, K Meagher, Y Luo… - Journal of the American Society of Nephrology, 2021