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DNA J homolog subfamily B member 9 and other advances in fibrillary glomerulonephritis.
Current Opinion in Nephrology and Hypertension ( IF 3.2 ) Pub Date : 2021-3-27 , DOI: 10.1097/mnh.0000000000000706
Nicole K Andeen 1 , Rupali S Avasare 2
Affiliation  

Fibrillary glomerulonephritis (FGN) involves ∼1% of native kidney biopsies and is characterized by glomerular deposition of fibrils larger than amyloid (12-24 nm diameter) composed of polyclonal immunoglobulin G (IgG). The recent discovery of DNA J homolog subfamily B member 9 (DNAJB9) in FGN glomerular deposits has contributed a specific and sensitive biomarker, informing morphologic classification and pathogenesis. This review will consider contemporary FGN incidence and genetics, pathogenesis, (lack of) paraprotein association, variants, treatment, and transplantation.

中文翻译:

DNA J 同源物 B 亚家族成员 9 和纤维性肾小球肾炎的其他进展。

纤维性肾小球肾炎 (FGN) 涉及约 1% 的天然肾活检,其特征是肾小球沉积比由多克隆免疫球蛋白 G (IgG) 组成的淀粉样蛋白(直径 12-24 nm)更大的原纤维。最近在 FGN 肾小球沉积物中发现 DNA J 同源亚家族 B 成员 9 (DNAJB9),这提供了一种特异性且敏感的生物标志物,为形态学分类和发病机制提供信息。本综述将考虑当代 FGN 的发病率和遗传学、发病机制、(缺乏)副蛋白关联、变异、治疗和移植。
更新日期:2021-03-30
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