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Metabolism. 1983 Feb;32(2):160-4.

Formation of beta-hydroxyisovalerate by an alpha-ketoisocaproate oxygenase in human liver.


Previously we demonstrated the occurrence of a soluble dioxygenase in rat liver which converts alpha-ketoisocaproic acid (the keto acid analog of leucine) to beta-hydroxyisovaleric acid. Herein we show that human liver contains a similar soluble enzyme which converts alpha-ketoisocaproate to beta-hydroxisovaleric acid. We suggest this enzyme functions as a "safety valve" in liver to help prevent excessive accumulation of alpha-ketoisocaproate.

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