Category:natural substances and extractives
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Notes: 3-methylbutyrolactone is lactone of 4-hydroxyisovaleric acid and it has been found in the urine of patients with isovaleric acidemia, an autosomal recessive genetic disorder of the enzyme isovaleryl-CoA dehydrogenase (PMID 6452974; PMID 6616835). Isovaleric acidemia is closely related to the genetic metabolic disorder Maple syrup urine disease. [HMDB]
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