Scholarlink

Background <p>Increased serum anti-nephrin antibody titers and co-localization of nephrin and IgG in kidney tissues have been reported in minimal change disease (MCD) and post-transplant recurrent focal segmental glomerulosclerosis (FSGS). These results indicate an association of anti-nephrin antibodies with nephrotic syndrome (NS); however, the exact relationship remains unclear. Herein, we evaluated nephrin/IgG co-localization in the glomeruli of patients with various kidney diseases, including monogenic NS, to clarify the association between idiopathic nephrotic syndrome (INS) and anti-nephrin antibodies.</p> Methods <p>IgG and nephrin co-localization was investigated in 52 kidney tissue biopsy samples, comprising INS in the active phase (<i>n</i> = 26; MCD, <i>n</i> = 19; FSGS, <i>n</i> = 7) and remission (<i>n</i> = 6), monogenic NS (<i>n</i> = 3), and other kidney diseases (<i>n</i> = 17). Double-immunofluorescence staining for nephrin/IgG was performed in unfixed frozen sections for 2&#xa0;h at room temperature with Alexa Fluor-labeled nephrin/IgG cocktail antibodies. Nephrin/IgG co-localization was assessed using optical sectioning under a fluorescence microscope.</p> Results <p>Nephrin/IgG co-localization was observed in 81% (21/26, children: 15/17, adults: 6/9) of active INS cases, 84% (16/19) of MCD cases, and 71% (5/7) of FSGS cases. No co-localization was observed in NS with monogenic variants or other kidney diseases.</p> Conclusion <p>Nephrin/IgG co-localization in the kidney tissue is finding observed in active INS<b>,</b> strongly indicating an association between anti-nephrin antibodies and INS onset. The nephrin/IgG cocktail antibody is a rapid and effective approach for investigating INS pathogenesis that facilitates the differential diagnosis of immune-mediated NS from other kidney diseases, including monogenic NS.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Co-localization of IgG with nephrin in immune-mediated idiopathic nephrotic syndrome

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2025年8月6日

查看全文
  • Yuta Ichikawa,
  • Nana Sakakibara,
  • Shuhei Aoyama,
  • Yuka Kimura,
  • Yuta Inoki,
  • Yu Tanaka,
  • Chika Ueda,
  • Hideaki Kitakado,
  • China Nagano,
  • Tomohiko Yamamura,
  • Shingo Ishimori,
  • Yuko Shima,
  • Hayaki Okamoto,
  • Hideki Fujii,
  • Hironobu Maruyama,
  • Kazumoto Iijima,
  • Kandai Nozu,
  • Tomoko Horinouchi

摘要

Background

Increased serum anti-nephrin antibody titers and co-localization of nephrin and IgG in kidney tissues have been reported in minimal change disease (MCD) and post-transplant recurrent focal segmental glomerulosclerosis (FSGS). These results indicate an association of anti-nephrin antibodies with nephrotic syndrome (NS); however, the exact relationship remains unclear. Herein, we evaluated nephrin/IgG co-localization in the glomeruli of patients with various kidney diseases, including monogenic NS, to clarify the association between idiopathic nephrotic syndrome (INS) and anti-nephrin antibodies.

Methods

IgG and nephrin co-localization was investigated in 52 kidney tissue biopsy samples, comprising INS in the active phase (n = 26; MCD, n = 19; FSGS, n = 7) and remission (n = 6), monogenic NS (n = 3), and other kidney diseases (n = 17). Double-immunofluorescence staining for nephrin/IgG was performed in unfixed frozen sections for 2 h at room temperature with Alexa Fluor-labeled nephrin/IgG cocktail antibodies. Nephrin/IgG co-localization was assessed using optical sectioning under a fluorescence microscope.

Results

Nephrin/IgG co-localization was observed in 81% (21/26, children: 15/17, adults: 6/9) of active INS cases, 84% (16/19) of MCD cases, and 71% (5/7) of FSGS cases. No co-localization was observed in NS with monogenic variants or other kidney diseases.

Conclusion

Nephrin/IgG co-localization in the kidney tissue is finding observed in active INS, strongly indicating an association between anti-nephrin antibodies and INS onset. The nephrin/IgG cocktail antibody is a rapid and effective approach for investigating INS pathogenesis that facilitates the differential diagnosis of immune-mediated NS from other kidney diseases, including monogenic NS.