Characterization of glomerular basement membrane components within pediatric glomerular diseases

作者全名："Chen, Dan; Zhou, Xindi; Gan, Chun; Yang, Qing; Chen, Wanbing; Feng, Xiaoqian; Zhang, Tao; Zhang, Li; Dai, Lujun; Chen, Yaxi; Yang, Haiping; Wang, Mo; Jiang, Wei; Li, Qiu"

作者地址："[Chen, Dan; Zhou, Xindi; Gan, Chun; Yang, Qing; Chen, Wanbing; Feng, Xiaoqian; Yang, Haiping; Wang, Mo; Jiang, Wei; Li, Qiu] Natl Clin Res Ctr Child Hlth & Disorders, Childrens Hosp, Chongqing Key Lab Pediat Metab & Inflammatory Dis, Minist Educ,Key Lab Child Dev & Disorders,Dept Nep, Chongqing, Peoples R China; [Feng, Xiaoqian] Chongqing Univ, Chongqing Univ Three Gorges Hosp, Sch Med, Chongqing, Peoples R China; [Zhang, Tao; Zhang, Li] Guizhou Med Univ, Affiliated Hosp, Guizhou Prov Childrens Med Ctr, Pediat Renal Immunol Specialist Sect, Guiyang, Guizhou, Peoples R China; [Dai, Lujun] Guizhou Med Univ, Affiliated Hosp, Dept Pathol, Guiyang, Guizhou, Peoples R China; [Chen, Yaxi] Chongqing Med Univ, Affiliated Hosp 2, Ctr Lipid Res, Chongqing, Peoples R China; [Chen, Yaxi] Chongqing Med Univ, Affiliated Hosp 2, Dept Infect Dis, Key Lab Mol Biol Infect Dis,Minist Educ,Inst Viral, Chongqing, Peoples R China"

通信作者："Jiang, W; Li, Q (通讯作者)，Natl Clin Res Ctr Child Hlth & Disorders, Childrens Hosp, Chongqing Key Lab Pediat Metab & Inflammatory Dis, Minist Educ,Key Lab Child Dev & Disorders,Dept Nep, Chongqing, Peoples R China."

来源：CLINICAL KIDNEY JOURNAL

ESI学科分类：CLINICAL MEDICINE

WOS号：WOS:001180941200002

JCR分区：Q1

影响因子：3.9

年份：2024

卷号：17

期号：3

开始页：　

结束页：　

文献类型：Article

关键词：collagen IV alpha 3 alpha 4 alpha 5; glomerular basement membrane; glomerular disease; integrin alpha 3 beta 1; laminin alpha 5 beta 2 gamma 1

摘要："Background. Disruptions in gene expression associated with the glomerular basement membrane (GBM) could precipitate glomerular dysfunction. Nevertheless, a comprehensive understanding of the characterization of GBM components within pediatric glomerular diseases and their potential association with glomerular function necessitates further systematic investigation. Methods. We conducted a systematic analysis focusing on the pathological transformations and molecular attributes of key constituents within the GBM, specifically Collagen IV alpha 3 alpha 4 alpha 5, Laminin alpha 5 beta 2 gamma 1, and Integrin alpha 3 beta 1, across prevalent pediatric glomerular diseases. Results. We observed upregulation of linear expression levels of COL4A3/4/5 and Laminin 5 alpha proteins, along with a partial reduction in the linear structural expression of Podocin in idiopathic nephrotic syndrome (INS), encompassing minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS), but showing a reduction in IgA nephropathy (IgAN), IgA vasculitis nephritis (IgAVN) and lupus nephritis (LN). Furthermore, our study revealed reductions in Laminin beta 2 gamma 1 and Integrin alpha 3 beta 1 in both primary and secondary childhood glomerular diseases. Conclusion. In INS, notably MCD and FSGS, there is a notable increase in the linear expression levels of COL4A3/4/5 and Laminin 5 alpha proteins. In contrast, in IgAN, IgAVN, and LN, there is a consistent reduction in the expression of these markers. Furthermore, the persistent reduction of Laminin beta 2 gamma 1 and Integrin alpha 3 beta 1 in both primary and secondary childhood glomerular diseases suggests a shared characteristic of structural alterations within the GBM across these conditions."

基金机构：Chongqing Natural Science Foundation; Department of Nephrology at the Children's Hospital of Chongqing Medical University; Urology and Nephropathy Center of the Second Affiliated Hospital of Chongqing Medical University

基金资助正文：Special thanks to the Department of Nephrology at the Children's Hospital of Chongqing Medical University for their assistance in performing kidney biopsies and providing clinical data. We would also like to thank the Urology and Nephropathy Center of the Second Affiliated Hospital of Chongqing Medical University for providing adjacent normal kidney tissue samples as healthy controls.