Document Detail

Renal Tubular Dysgenesis and Tubulointerstitial Nephritis Antigen in Juvenile Nephronophthisis.
MedLine Citation:
PMID:  21265929     Owner:  NLM     Status:  Publisher    
Introduction: We evaluated the relationship between abnormalities of tubular architecture and tubulointerstitial nephritis antigen (TIN-ag) in juvenile nephronophthisis (J-NPH). Method: 16 J-NPH patients were examined. Nephrocystin-1, TIN-ag, and type IV collagen, Fas antigen, and the C5b-9 complement complex were stained by immunohistochemical methods. Results: Renal tubules of patients with J-NPH showed morphologic abnormalities of tubular basement membranes (TBM) and frequent apoptosis of tubular epithelial cells. Additionally, C5b-9 complement complex was deposited within the TBM in the absence of immunoglobulin deposition, suggesting complement-dependent TBM injury. Localization of TIN-ag in the TBM of J-NPH patients disclosed a partial defect or discontinuity in 14 of 16 patients, while type IV collagen immunoreactivity was relatively preserved. These findings suggest that in J-NPH patients, tubulogenesis is disturbed during nephronogenesis because of a defect in nephrocystin, an NPHP gene product. TBM defects induce further morphologic abnormalities such as cystic dilation of tubules; as tubular function impairment advances, the incomplete tubules may be injured by C5b-9 complement complexes, followed by apoptotic cell death. Conclusion: TIN-ag, which is important early nephrogenesis, lacks normal activity, and vulnerable and incomplete tubules with deficient TIN-ag expression are formed. Removal of these defective tubules by apoptosis combined with the C5b-9 complement complex could be the primary reason for progression to ESRD in J-NPH patients.
Keisuke Sugimoto; Yutaka Takemura; Hidehiko Yanagida; Shinsuke Fujita; Tomoki Miyazawa; Naoki Sakata; Mitsuru Okada; Tsukasa Takemura
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-1-26
Journal Detail:
Title:  Nephrology (Carlton, Vic.)     Volume:  -     ISSN:  1440-1797     ISO Abbreviation:  -     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-1-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9615568     Medline TA:  Nephrology (Carlton)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2011 The Authors. Nephrology © 2011 Asian Pacific Society of Nephrology.
Department of Pediatrics, Kinki University School of Medicine, Osaka, Japan.
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