Isovalerylglycine
National Institutes of Health
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Isovaleric acidemia, resulting from isovaleryl-coenzyme A dehydrogenase deficiency, is associated with marked reduction of free…
Isovaleric Acidemia is treated with L-leucine restriction and supplemental glycine. Added glycine augments conversion of…
Isovaleric acidemia secondary to isovaleryl-CoA dehydrogenase deficiency is a rare disorder of organic acid metabolism occuring…
Three patients are described with different forms of isovaleric acidemia: a girl with the neonatal form and two brothers with an…
Urine from one of the original patients with butyric and hexanoic aidemia was examined for the possible excretion of glycine…