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01-06-2009 | Original Paper

Primary cultures of renal proximal tubule cells derived from individuals with primary hyperoxaluria

Authors: Karen L. Price, Sally-Anne Hulton, William G. van’t Hoff, John R. Masters, Gill Rumsby

Published in: Urolithiasis | Issue 3/2009

Abstract

The primary hyperoxalurias, PH1 and PH2, are inherited disorders caused by deficiencies of alanine:glyoxylate aminotransferase and glyoxylate reductase, respectively. Mutations in either of these enzymes leads to endogenous oxalate overproduction primarily in the liver, but most pathological effects are exhibited in the kidney ultimately leading to end-stage renal failure and systemic oxalosis. To provide a non-invasive means of accessing kidney cells from individuals with primary hyperoxaluria, we have derived primary cultures of renal proximal tubule cells from the urine of these patients. The cells stain positively for the epithelial markers pan-cytokeratin and zonula occludens 1 and the proximal tubule marker γ-glutamyl transpeptidase. Mutation analysis confirmed that the cultured cells had the same genotype as the leucocytes of the patients and also expressed glyoxylate reductase at the mRNA level, illustrating their potential value as a source of renal material from these individuals.

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Title: Primary cultures of renal proximal tubule cells derived from individuals with primary hyperoxaluria
Authors: Karen L. Price
Sally-Anne Hulton
William G. van’t Hoff
John R. Masters
Gill Rumsby
Publication date: 01-06-2009
Publisher: Springer-Verlag
Published in: Urolithiasis / Issue 3/2009
Print ISSN: 2194-7228
Electronic ISSN: 2194-7236
DOI: https://doi.org/10.1007/s00240-009-0185-5

At a glance: The STEP trials

Springer Medicine

Primary cultures of renal proximal tubule cells derived from individuals with primary hyperoxaluria

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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