Document Detail


Renal Tubular Dysgenesis and Tubulointerstitial Nephritis Antigen in Juvenile Nephronophthisis.
MedLine Citation:
PMID:  21265929     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Keisuke Sugimoto; Yutaka Takemura; Hidehiko Yanagida; Shinsuke Fujita; Tomoki Miyazawa; Naoki Sakata; Mitsuru Okada; Tsukasa Takemura
Related Documents :
16601919 - Negative correlation between bone mineral density and tsh receptor antibodies in male p...
17317589 - Hematopoietic cell transplantation as curative therapy for idiopathic myelofibrosis, ad...
6681149 - Protrusio acetabuli in osteoporosis and osteomalacia.
2340659 - Evaluation of intense renal parenchymal activity ("hot kidneys") on bone scintigraphy.
11453239 - Retrospective evaluation of the influence of the interleukin-1 genotype on radiographic...
11261329 - Ineffectiveness of interferon-gamma in the treatment of idiopathic myelofibrosis: a pil...
23650469 - Vitamin k antagonist warfarin for palliative treatment of metachromatic leukodystrophy,...
21514679 - Pediatric otogenic lateral sinus thrombosis recanalization.
1758729 - Efficacy, results, and complications of mechanical ventilation in children with status ...
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.
Affiliation:
Department of Pediatrics, Kinki University School of Medicine, Osaka, Japan.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Teaching pathology via online digital microscopy: Positive learning outcomes for rurally based medic...
Next Document:  Fibroblast growth factor 23 in chronic kidney disease: new insights and clinical implications.