Document Detail

Elevated oxidized glutathione in cystinotic proximal tubular epithelial cells.
MedLine Citation:
PMID:  16202976     Owner:  NLM     Status:  MEDLINE    
Cystinosis, the most frequent cause of inborn Fanconi syndrome, is characterized by the lysosomal cystine accumulation, caused by mutations in the CTNS gene. To elucidate the pathogenesis of cystinosis, we cultured proximal tubular cells from urine of cystinotic patients (n = 9) and healthy controls (n = 9), followed by immortalization with human papilloma virus (HPV E6/E7). Obtained cell lines displayed basolateral polarization, alkaline phosphatase activity, and presence of aminopeptidase N (CD-13) and megalin, confirming their proximal tubular origin. Cystinotic cell lines exhibited elevated cystine levels (0.86 +/- 0.95 nmol/mg versus 0.09 +/- 0.01 nmol/mg protein in controls, p = 0.03). Oxidized glutathione was elevated in cystinotic cells (1.16 +/- 0.83 nmol/mg versus 0.29 +/- 0.18 nmol/mg protein, p = 0.04), while total glutathione, free cysteine, and ATP contents were normal in these cells. In conclusion, elevated oxidized glutathione in cystinotic proximal tubular epithelial cell lines suggests increased oxidative stress, which may contribute to tubular dysfunction in cystinosis.
Martijn J G Wilmer; Adriana de Graaf-Hess; Henk J Blom; Henry B P M Dijkman; Leo A Monnens; Lambertus P van den Heuvel; Elena N Levtchenko
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2005-09-23
Journal Detail:
Title:  Biochemical and biophysical research communications     Volume:  337     ISSN:  0006-291X     ISO Abbreviation:  Biochem. Biophys. Res. Commun.     Publication Date:  2005 Nov 
Date Detail:
Created Date:  2005-10-14     Completed Date:  2005-12-21     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0372516     Medline TA:  Biochem Biophys Res Commun     Country:  United States    
Other Details:
Languages:  eng     Pagination:  610-4     Citation Subset:  IM    
Laboratory of Pediatrics and Neurology, Radboud University Nijmegen Medical Centre, The Netherlands.
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MeSH Terms
Adenosine Triphosphate / metabolism
Antigens, CD13 / metabolism
Cells, Cultured
Cystine / metabolism
Cystinosis / metabolism,  pathology*
Epithelial Cells / cytology,  metabolism*
Glutathione Disulfide / metabolism*
Kidney Tubules, Proximal / metabolism,  pathology*
LDL-Receptor Related Protein 2 / metabolism
Reg. No./Substance:
0/LDL-Receptor Related Protein 2; 27025-41-8/Glutathione Disulfide; 56-65-5/Adenosine Triphosphate; 56-89-3/Cystine; EC, CD13

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