Template:Leucine metabolism in humans

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Human metabolic pathway for HMB and isovaleryl-CoA relative to L-leucine.[3][4][5] Of the two major pathways, L-leucine is mostly metabolized into isovaleryl-CoA, while only about 5% is metabolized into HMB.[3][4][5]
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References

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  1. ↑ "KEGG Reaction: R10759". Kanehisa Laboratories. http://www.genome.jp/dbget-bin/www_bget?rn:R10759. Retrieved 24 June 2016. 
  2. ↑ "Urinary excretion of 3-hydroxyisovaleric acid and 3-hydroxyisovaleryl carnitine increases in response to a leucine challenge in marginally biotin-deficient humans". The Journal of Nutrition 141 (11): 1925–1930. November 2011. doi:10.3945/jn.111.146126. PMID 21918059. "Metabolic impairment diverts methylcrotonyl CoA to 3-hydroxyisovaleryl CoA in a reaction catalyzed by enoyl-CoA hydratase (22, 23). 3-Hydroxyisovaleryl CoA accumulation can inhibit cellular respiration either directly or via effects on the ratios of acyl CoA:free CoA if further metabolism and detoxification of 3-hydroxyisovaleryl CoA does not occur (22). The transfer to carnitine by 4 carnitine acyl-CoA transferases distributed in subcellular compartments likely serves as an important reservoir for acyl moieties (39–41). 3-Hydroxyisovaleryl CoA is likely detoxified by carnitine acetyltransferase producing 3HIA-carnitine, which is transported across the inner mitochondrial membrane (and hence effectively out of the mitochondria) via carnitine-acylcarnitine translocase (39). 3HIA-carnitine is thought to be either directly deacylated by a hydrolase to 3HIA or to undergo a second CoA exchange to again form 3-hydroxyisovaleryl CoA followed by release of 3HIA and free CoA by a thioesterase.". 
  3. ↑ 3.0 3.1 "International Society of Sports Nutrition Position Stand: beta-hydroxy-beta-methylbutyrate (HMB)". Journal of the International Society of Sports Nutrition 10 (1): 6. February 2013. doi:10.1186/1550-2783-10-6. PMID 23374455. 
  4. ↑ 5.0 5.1 Nutrient Metabolism: Structures, Functions, and Genes (2nd ed.). Academic Press. May 2015. pp. 385–388. ISBN 978-0-12-387784-0. https://books.google.com/books?id=aTQTAAAAQBAJ&printsec=frontcover#v=onepage. Retrieved 6 June 2016. "Energy fuel: Eventually, most Leu is broken down, providing about 6.0kcal/g. About 60% of ingested Leu is oxidized within a few hours ... Ketogenesis: A significant proportion (40% of an ingested dose) is converted into acetyl-CoA and thereby contributes to the synthesis of ketones, steroids, fatty acids, and other compounds" 
    Figure 8.57: Metabolism of L-leucine
Notes
  1. ↑ This reaction is catalyzed by an unknown thioesterase enzyme.[1][2]
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