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【包邮】2–Hydroxy–3–methylbutanoic?acid
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产品编号 GC31222
2–Hydroxy–3–methylbutanoic acid (3–Methyl–2–hydroxybutyric acid, 2–Hydroxyisopentanoic acid, 2–Oxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria, methylmalonic acidemia, propionic acidemia, 3–ketothiolase deficiency, isovaleric acidemia, 3–methylcrotonylglycemia, 3–hydroxy–3–methylglutaric acidemia, multiple carboxylase deficiency, glutaric aciduria, ornithine transcarbamylase deficiency, glyceroluria, tyrosinemia type I, galactosemia, and maple syrup urine disease·
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