摘要
IgA肾病(IgA nephropathy,IgAN)是儿童及青少年最常见的原发性肾小球肾炎,是慢性肾脏病和终末期肾病的主要原因。目前IgAN发病机制尚未完全清楚,可能与多重免疫打击有关。即半乳糖缺陷型IgA1(galactose-deficient IgA1,Gd-IgA1)形成(第一重打击);抗Gd-IgA1的自身抗体合成(第二重打击);Gd-IgA1与自身抗体结合形成循环免疫复合物(第三重打击);这些含有Gd-IgA1的循环免疫复合物在肾小球系膜中沉积,引发肾脏损害(第四重打击)。Gd-IgA1是IgAN发生发展始发及驱动的关键因素,核心1β1,3-半乳糖基转移酶(core 1,β1,3-galactosyltransferase,C1GALT1)是IgA1 O-糖基化过程中的关键酶,其表达减少和(或)活性下降与Gd-IgA1的产生密切相关。该综述阐述了C1GALT1在IgAN发病、治疗及预后中的作用。
IgA nephropathy(IgAN)is the most common primary glomerulonephritis in children and adolescents.It is an important cause of chronic kidney disease and end stage renal disease.The pathogenesis of IgAN has not been fully elucidated and it is thought to be associated with a multi-hit hypothesis,namely,increased levels of galactose-deficient IgA1(Gd-IgA1)(Hit 1);production of auto-antibodies directed against Gd-IgA1(Hit 2);formation of Gd-IgA1-containing immune complexes(Hit 3);the deposition of immune complexes in the glomerular mesangium resulting in glomerular injury(Hit 4).Gd-IgA1 is regarded as the initiator of the pathogenesis of IgAN.Core 1,β1,3-galactosyltransferase(C1GALT1)is a key enzyme in the process of O-glycosylation of IgA.The reduction in the activity and/or gene expression of C1GALT1 is closely related to Gd-IgA1.This review will illustrate the role of C1GALT1 in the pathogenesis,diagnosis,treatment and prognosis of IgAN to provide molecular strategies for the clinical practice.
作者
刘莹
刘彩琼(综述)
赵明一
何庆南(审校)
Liu Ying;Liu Caiqiong;Zhao Mingyi;He Qingnan(Department of Pediatrics,the Third Xiangya Hospital of Central South University,Changsha 410013,China;Department of Pediatric Nephrology,Children′s Medical Center,the Second Xiangya Hospital,Central South University,Changsha 410011,China)
出处
《国际儿科学杂志》
2020年第9期636-639,共4页
International Journal of Pediatrics
基金
国家自然科学基金(81970248)。
作者简介
通信作者:何庆南,Email:heqn2629@csu.edu.cn。
