The role of C1GALT1 in the pathogenesis of IgA nephropathy / 国际儿科学杂志

ABSTRACT

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.