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      Human liver L-alanine-glyoxylate aminotransferase: characteristics and activity in controls and hyperoxaluria type I patients using a simple spectrophotometric method.

      Clinica Chimica Acta; International Journal of Clinical Chemistry

      Transaminases, blood, Alanine Transaminase, Biopsy, Glyoxylates, Humans, Alanine, Hydrogen-Ion Concentration, Hyperoxaluria, enzymology, Kinetics, Liver, Spectrophotometry

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          Abstract

          We have studied the characteristics of human liver alanine-glyoxylate aminotransferase, which is deficient in hyperoxaluria type I, an inherited disorder of glyoxylate metabolism. The enzyme was optimally active at pH 8.0 showing apparent Km values for L-alanine and glyoxylate of 8.3 and 1.3 mmol/l, respectively. Activity was found to proceed linearly for up to 4 h. Measurements under these optimal conditions enabled the biochemical diagnosis of hyperoxaluria type I to be made via enzyme activity measurements in percutaneous needle biopsy specimens of liver tissue.

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          2397596

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