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      Peroxisomal alanine:glyoxylate aminotransferase deficiency in primary hyperoxaluria type I.

      Febs Letters

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

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          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.

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          3709805

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