title: Formation of beta-hydroxyisovalerate by an alpha-ketoisocaproate oxygenase in human liver.
abstract: 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.
mesh_terms: D055598:Chemical Phenomena; D002621:Chemistry; D049308:Dioxygenases; D006801:Humans; D066298:In Vitro Techniques; D008099:Liver / Q000201:enzymology*; D010105:Oxygenases / Q000378:metabolism*; D012995:Solubility; D014631:Valerates / Q000096:biosynthesis*
chemical_list: D014631:Valerates; C004961:beta-hydroxyisovaleric acid; D010105:Oxygenases; D049308:Dioxygenases; C034776:alpha-ketoisocaproate oxygenase