Peroxisomal alanine:glyoxylate aminotransferase deficiency in primary hyperoxaluria type I

FEBS Lett. 1986 May 26;201(1):20-4. doi: 10.1016/0014-5793(86)80563-4.

Abstract

Activities of alanine:glyoxylate aminotransferase in the livers of two patients with primary hyperoxaluria type I were substantially lower than those found in five control human livers. Detailed subcellular fractionation of one of the hyperoxaluric livers, compared with a control liver, showed that there was a complete absence of peroxisomal alanine:glyoxylate aminotransferase. This enzyme deficiency explains most of the biochemical characteristics of the disease and means that primary hyperoxaluria type I should be added to the rather select list of peroxisomal disorders.

MeSH terms

  • Alanine Transaminase / deficiency*
  • Centrifugation, Density Gradient
  • Glycolates / urine
  • Humans
  • Liver / enzymology*
  • Microbodies / enzymology*
  • Oxalates / urine*
  • Oxalic Acid
  • Subcellular Fractions / enzymology
  • Transaminases*

Substances

  • Glycolates
  • Oxalates
  • glycolic acid
  • Oxalic Acid
  • Transaminases
  • Alanine Transaminase
  • Alanine-glyoxylate transaminase