Article
Changes in glomerular mesangium in kidneys with congenital nephrotic syndrome of the Finnish type.
Pediatric nephrology (Berlin, Germany) - 1 May 2010
Kaukinen Anne, Kuusniemi Arvi-Matti, Helin Heikki, Jalanko Hannu
Abstract excerpt
Congenital nephrotic syndrome of the Finnish type (NPHS1, CNF) is an autosomal recessive disease caused by mutations in a major podocyte protein, nephrin. NPHS1 is associated with heavy proteinuria and the development of glomerular scarring. We studied the cellular and molecular changes affecting the glomerular mesangium in NPHS1 kidneys. Marked hyperplasia of mesangial cells (MC) was mainly responsible for the...
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