Aminoaciduria

Abstract
Review with 208 references. Metabolic disorders reviewed include disorders of amino acid metabolism (phenylketonuria, tyrosinosis, histidinemia, imidazole amlnoaciduria, maple syrup urine disease: branched chain ketoaciduria, hypervallnemia, hyserglycinemia, and hypersarcosinemia); disorders of imino acid metabolism (hyperprollnemia and hydroxyprollnemla); disorders of sulfur-containing amino acids (cystathioninuria, homocystinuria, and methioninemia) disorders of urea cycle enzymes (argininoosuccinic aciduria, citrul-linemia, hyperammonemia, and hyperlysinemia); and disorders of amino acid transport (cystinuria, Hartnup disease, and generalized renal aminoaciduria).

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