Podocytopathy and Nephrotic Syndrome in Mice with Podocyte-Specific Deletion of the Asah1 Gene
作者: Guangbi LiJason KiddCristin KasparSara DempseyOwais M. BhatSarah CamusJoseph K. RitterTodd W.B. GehrErich GulbinsPin-Lan Li
作者单位: 1Department of Pharmacology and Toxicology, Virginia Commonwealth University, Richmond, Virginia
2Division of Nephrology, School of Medicine, Virginia Commonwealth University, Richmond, Virginia
3Department of Molecular Biology, University of Duisburg-Essen, Essen, Germany
刊名: The American Journal of Pathology, 2020, Vol.190 (6), pp.1211-1223
来源数据库: Elsevier Journal
DOI: 10.1016/j.ajpath.2020.02.008
英文摘要: Lysosomal acid ceramidase (Ac) has been shown to be critical for ceramide hydrolysis and regulation of lysosome function and cellular homeostasis. In the present study, we generated a knockout mouse strain ( Asah1 fl/fl /Podo Cre ) with a podocyte-specific deletion of the α subunit (main catalytic subunit) of Ac. Although no significant morphologic changes in glomeruli were observed in these mice under light microscope, severe proteinuria and albuminuria were found in these podocyte-specific knockout mice compared with control genotype littermates. Transmission electron microscopic analysis showed that podocytes of the knockout mice had distinctive foot process effacement and microvillus formation. These functional and morphologic changes indicate the development of nephrotic syndrome in...
全文获取路径: Elsevier  (合作)
影响因子:4.522 (2012)