Culture of renal tubular cells from the urine of patients with nephropathic cystinosis

Lorraine C. Racusen, Barbara A. Fivush, Hans Andersson, William A. Gahl

Research output: Contribution to journalArticle

Abstract

Nephropathic cystinosis represents a prototype for lysosomal storage diseases and is the most common cause of renal tubular Fanconi's syndrome. Mechanisms of the tubular transport defects in this disease have not been defined, however, in part because the cells readily cultured from affected patients, leukocytes and fibroblasts, do not express epithelial transport functions. Except for a single autopsy report, renal tubular cells from these patients have not been studied In vitro. In these studies, noninvasive harvesting and culture of renal tubular cells from the urine of patients with cystinosis is described. Cultures of renal tubular cells could be established from over 50% of the isolates which contained viable cells and which remained uncontaminated in vitro. Cells had an epithelial morphology in culture, and the majority of cultured cells expressed proximal tubular brush border marker enzyme. Cultured cells also expressed the storage defect in vitro, containing cystine levels up to 100 times those of normal cells. Cultured cells could be depleted of cystine by using the thiol cysteamine. This in vitro model system should be very useful for studying the mechanisms of renal tubular transport defects in this disease.

Original languageEnglish (US)
Pages (from-to)1028-1033
Number of pages6
JournalJournal of the American Society of Nephrology
Volume1
Issue number8
StatePublished - Feb 1991

Keywords

  • Cysteamine
  • Cystine
  • Kidney
  • Lysosome
  • Storage disease

ASJC Scopus subject areas

  • Nephrology

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