Clinica chimica acta; international journal of clinical chemistry
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alpha-Mannosidase and beta-N-acetylglucosaminidase activities were found to differ between lymphocytes and granulocytes isolated from bovine blood. Activities of both enzymes in granulocyte preparations were found to be related to the eosinophil content of the preparations. A procedure is described that allows definition of the mannosidosis genotype of adult cattle by giving consideration to the influence of eosinophil content of granulocyte preparations upon the activity of alpha-mannosidase relative to that of beta-N-acetylglucosaminidase.
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In 62 blood samples from 3 patients with classical maple syrup urine disease and from one patient with a variant form, a close linear correlation was found between levels of branched chain amino acids and their corresponding alpha-keto acids. Keto acids were determined as O-trimethylsilyl quinoxalinols by gas chromatography with a nitrogen-selective detector.