Abstract
In last year’s introductory volume to this series, we indicated that our strategy in these annual surveys of amino acid and organic acid metabolism would involve detailed discussion of a few topics rather than cursory treatment of many. We have maintained that posture this year. Three topics have been chosen for discussion: phenylketonuria and its variants, the γ-glutamyl cycle and 5-oxoprolinuria, and Jamaican vomiting sickness and glutaric aciduria. These areas concern “old” (phenylketonuria) and “new” (5-oxoprolinuria) diseases, common (Jamaican vomiting sickness) and rare (glutaric aciduria) ones. Although the metabolic pathways chosen for discussion are widely different, the scientific and clinical signposts are familiar. They include considerations of biochemical and genetic heterogeneity, evaluation of diagnostic and therapeutic modalities, the convergence of information gained from related acquired and inherited abnormalities, and, in all instances, the existence of important, unanswered questions. We have attempted to cite only major, recent references individually, and have depended on reviews in each section to provide the interested reader with older but clearly not lesser works. In this way, we hope that involved workers and serious readers will gain the flavor of the field without much in the way of a bitter or sour aftertaste.
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Rosenberg, L.E., Tanaka, K. (1978). Metabolism of Amino Acids and Organic Acids. In: Freinkel, N. (eds) The Year in Metabolism 1977. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-2499-7_7
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