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Identification of the Source of Secreted Proteins in the Kidney by Brefeldin A Injection
Published on: November 10, 2021
Transforming growth factor-beta in renal disease with glycogen storage disease I
Maki Urushihara1, Shoji Kagami, Michinori Ito
1Department of Pediatrics, School of Medicine, University of Tokushima, and Division of Pediatrics, Tokushima Red Cross Hospital, Komatsushima City, Japan. urushi@tokushima-med.jrc.or.jp
Abstract:
We report a 14-year-old patient with Japanese glycogen storage disease I (GSD-I) who was found to have proteinuria. Renal biopsy revealed massive tubular atrophy and interstitial fibrosis with mononuclear cell infiltration, but the glomeruli were almost normal. The epithelial cells of tubules contained periodic acid-Schiff-positive glycogen deposits digested by diastase. In an immunohistological study, transforming growth factor (TGF)-beta expression was increased in tubular epithelial cells compared with a normal control kidney specimen. These data suggest that increased TGF-beta expression is involved in the pathophysiology of renal interstitial fibrosis in a patient with GSD-I.
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