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Deficiency of Peroxisomal Alanine: Glyoxylate Aminotransferase in Primary Hyperoxaluria Type 1

  • C. J. Danpure
  • P. R. Jennings
Part of the Proceedings in Life Sciences book series (LIFE SCIENCES)

Abstract

The activity of alanine:glyoxylate aminotransferase (AGT) in the livers of two patients with primary hyperoxaluria type 1 (PH) was substantially lower than that found in six control human livers. Detailed subcellular fractionation of a pyridoxine-resistant PH liver, compared with a control liver, showed that there was a complete absence of peroxisomal AGT. The residual AGT activity in the PH liver could not be enhanced in vitro by excess pyridoxal phosphate. Unlike a number of other peroxisomal disorders, PH appears to be due to a specific enzyme deficiency with no evidence of a generalized perturbation of peroxisomal function.

Keywords

Calcium Oxalate Autosomal Recessive Disease Pyridoxal Phosphate Control Liver Primary Hyperoxaluria 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Berlin Heidelberg 1987

Authors and Affiliations

  • C. J. Danpure
    • 1
  • P. R. Jennings
    • 1
  1. 1.Division of Inherited Metabolic DiseasesClinical Research CentreMiddlesexUnited Kingdom

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