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  2. Methylmalonic acidemias - Wikipedia

    en.wikipedia.org/wiki/Methylmalonic_acidemias

    Methylmalonic acid metabolism including some methylmalonic acidemias. In methylmalonic acidemias, the body is unable to break down properly: essential amino acids: methionine, valine, threonine and isoleucine [39] propionic acid from intestinal fermentation [39] odd-chain fatty acids [39] cholesterol side chain [39]

  3. Organic acidemia - Wikipedia

    en.wikipedia.org/wiki/Organic_acidemia

    The branched-chain amino acids include isoleucine, leucine and valine. [1] Organic acids refer to the amino acids and certain odd-chained fatty acids which are affected by these disorders. The four main types of organic acidemia are: methylmalonic acidemia, propionic acidemia, isovaleric acidemia, and maple syrup urine disease. [1]

  4. Branched-chain amino acid - Wikipedia

    en.wikipedia.org/wiki/Branched-chain_amino_acid

    A branched-chain amino acid (BCAA) is an amino acid having an aliphatic side-chain with a branch (a central carbon atom bound to three or more carbon atoms). Among the proteinogenic amino acids, there are three BCAAs: leucine, isoleucine, and valine. [1] Non-proteinogenic BCAAs include 2-aminoisobutyric acid and alloisoleucine. Leucine ...

  5. Why Experts Say This Underrated Supplement Is Key To Building ...

    www.aol.com/why-experts-underrated-supplement...

    The Best Amino Acid Supplements To Try Amino acid complexes are available in powder form, but many brands also offer oral capsules. Choose a product that is third-party tested to ensure the ...

  6. Maple syrup urine disease - Wikipedia

    en.wikipedia.org/wiki/Maple_syrup_urine_disease

    The blood concentration of leucine and isoleucine is measured relative to other amino acids to determine if the newborn has a high level of branched-chain amino acids. Once the newborn is 2–3 days old the blood concentration of branched-chain amino acids like leucine is greater than 1000 μmol/L and alternative screening methods are used.

  7. Bodybuilding supplement - Wikipedia

    en.wikipedia.org/wiki/Bodybuilding_supplement

    The sources of protein are as follows and differ in protein quality depending on their amino acid profile and digestibility: Whey protein contains high levels of all the essential amino acids and branched-chain amino acids. It also has the highest content of the amino acid cysteine, which aids in the biosynthesis of glutathione.

  8. Branched-chain keto acid dehydrogenase kinase deficiency

    en.wikipedia.org/wiki/Branched-chain_keto_acid...

    Branched-chain keto acid dehydrogenase kinase deficiency (BCKDK deficiency) is a disease resulting from mutations of the BCKDK gene. Patients with BCKDK deficiency have low levels of branched chain amino acids (BCAA) in their organism due to accelerated breakdown of these essential amino acids.

  9. BCKDK - Wikipedia

    en.wikipedia.org/wiki/BCKDK

    BCKDK regulates the activity of branched-chain α-ketoacid dehydrogenase complex (BCKD) through phosphorylation and inactivation. This inactivation results in increased branched-chain amino acids (BCAA), which is seen to reduce oxidative stress; however, having too much BCAA has been proven to be toxic to humans. Therefore, BCKDK is a vital ...

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