期刊
AMERICAN JOURNAL OF KIDNEY DISEASES
卷 59, 期 2, 页码 284-292出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1053/j.ajkd.2011.07.024
关键词
Podocyte; proteinuria; glomerulus; nephrotic syndrome; N-acetyl-D-mannosamine (ManNAc); sialic acid
资金
- National Institute of Diabetes and Digestive and Kidney Diseases [R01 DK077073]
The pathogenesis of minimal change disease (MCD), considered to be the simplest form of nephrotic syndrome, has been one of the major unsolved mysteries in kidney disease. In this review, recent landmark studies that have led to the unraveling of MCD are discussed. A recent study now explains the molecular basis of major clinical and morphologic changes in MCD. Overproduction of angiopoietin-like 4 (ANGPTL4) in podocytes in MCD causes binding of ANGPTL4 to the glomerular basement membrane, development of nephrotic-range selective proteinuria, diffuse effacement of foot processes, and loss of glomerular basement membrane charge, but is not associated with changes shown by light microscopy in the glomerular and tubulointerstitial compartments. At least some of this ability of ANGPTL4 to induce proteinuria is linked to a deficiency of sialic acid residues because oral supplementation with sialic acid precursor N-acetyl-D-mannosamine improves sialylation of podocyte-secreted ANGPTL4 and significantly decreases proteinuria. Animal models of MCD, recent advances in potential biomarkers, and studies of upstream factors that may initiate glomerular changes also are discussed. In summary, recent progress in understanding MCD is likely to influence the diagnosis and treatment of MCD in the near future. Am J Kidney Dis. 59(2):284-292. (C) 2012 by the National Kidney Foundation, Inc.
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