Urological Research

, Volume 37, Issue 3, pp 127–132

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

  • Karen L. Price
  • Sally-Anne Hulton
  • William G. van’t Hoff
  • John R. Masters
  • Gill Rumsby
Original Paper

DOI: 10.1007/s00240-009-0185-5

Cite this article as:
Price, K.L., Hulton, SA., van’t Hoff, W.G. et al. Urol Res (2009) 37: 127. doi:10.1007/s00240-009-0185-5

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.

Keywords

Primary hyperoxaluria Kidney Human proximal tubular cell Cell culture 

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Karen L. Price
    • 1
  • Sally-Anne Hulton
    • 2
  • William G. van’t Hoff
    • 3
  • John R. Masters
    • 1
  • Gill Rumsby
    • 4
  1. 1.Institute of Urology and NephrologyUniversity College LondonLondonUK
  2. 2.Department of NephrologyBirmingham Children’s Hospital NHS TrustBirminghamUK
  3. 3.Nephro-Urology UnitUCL Institute of Child HealthLondonUK
  4. 4.Clinical BiochemistryUniversity College London HospitalsLondonUK

Personalised recommendations