a-Keto-b-Methylvaleric Acid is a B-Complex Vitamin Marker. Vitamins are compounds that your body needs to be healthy. Vitamins are “essential” for proper function, which means that they are not made inside your body and must be consumed in the diet. The B-complex vitamins are necessary for many enzymes in your body to function properly. Your body uses enzymes to extract energy from food, to build new tissue, to remove toxins, and to maintain the immune system. The branched-chain amino acids are broken down to form α-ketoisovalerate, α-ketoisocaproate, and α-keto-β-methylvalerate.
A dehydrogensase enzyme is needed for this step. Vitamins B1 , B2 , B3 , B5 , and lipoic acid are needed for this dehydrogenase to function properly. If these nutrients are insufficient, the keto acids may build up in the urine.
References:
- http://ctdbase.org/detail.go?type=chem&acc=C016211
- https://www.gdx.net/uk/core-uk/interpretive-guides-uk/Organix-IG.pdf
- http://www.oxfordreference.com/view/10.1093/oi/authority.20110803095405411
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a-Keto-b-Methylvaleric Acid comes from the essential amino acid isoleucine via transamination. Moderate elevations of a-Keto-b-Methylvaleric Acid usually mean that a-Keto-b-Methylvaleric Acid's further metabolism to the compound, alpha-methylbutyryl-CoA, is impaired either by coenzyme/cofactor insufficiency or by (genetic) weakness in the enzyme complex.
The dehydrogenase enzyme complex that accomplishes this requires lipoic acid, vitamin B1 as thiamin pyrophosphate and vitamin B2 as FAD. Vitamin B3 as NAD is a necessary cofactor, which removes hydrogen to become NADH. The other necessary cofactor is coenzyme A, requiring pantothenic acid, cysteine, and magnesium.
Elevated levels of a-Keto-b-Methylvaleric Acid may indicate low levels of any of these nutrients. The toxic elements arsenic, antimony, mercury and cadmium may also weaken lipoic acid and dehydrogenase activity.
Very high elevation of AKBM and its sister keto acids (alpha-ketoisovaleric, alpha-ketoisocaproic) constitute a rare disorder called "maple syrup urine disease".
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1-Methylhistidine, 1-Methylhistidine (Plasma), 2-,3-, and 4-Methylhippuric acid, 2-Methylhippuric Acid, 2-Methylsuccinic Acid, 3,4-Dihydroxyhydrocinnamic Acid, 3,5-Dihydroxybenzoic Acid, 3-Methylhistidine (Plasma), 3-Phenylpropionylglycine, 4-Hydroxybenzoic Acid, 4-Hydroxyphenylacetic Acid, 4-Hydroxyphenylpyruvic Acid, 5-Hydroxyindoleacetic Acid, 8-Hydroxy-2'-deoxyguanosine, a-Hydroxybutyric Acid, a-Keto-b-methylvaleric Acid, a-Ketobutyric Acid, a-Ketoglutaric Acid, a-Ketoisocaproic Acid, a-Ketoisovaleric Acid, a-Aminoadipic Acid (Plasma), Adipic Acid, Alanine, Alanine (Plasma), Aldosterone, Anserine (Plasma), Anthranilic Acid, Arabinitol, Arginine (Plasma), Arginosuccinic Acid, Arginosuccinic Acid (Plasma), Asparagine (Plasma), Aspartic Acid (Plasma), b-Hydroxybutyric Acid, b-Hydroxyisovaleric Acid, b-Alanine (Plasma), Benzoic Acid, Benzoylform, Branched Chain Alpha-Keto Organic Acids, Carnosine, Carnosine (Plasma), cis-Aconitic Acid, Citric Acid, Citrulline (Plasma), Cortisol, Cortisone, Creatinine, Cystathionine (Plasma), Cystine (Plasma), D-Lactic Acid, Equol, Ethanolamine (Plasma), Ethylmalonic Acid, Formiminoglutamic Acid, Fructose, Fumaric Acid, g-Aminobutyric Acid (Plasma), Glucaric Acid, Glucose, Glutamic Acid (Plasma), Glutamine (Plasma), Glutamine / Glutamate Ratio (Plasma), Glutaric Acid, Glycine (Plasma), Glycylproline (Plasma), Hexanoylglycine, Hippuric Acid, Histidine (Plasma), Homocitrulline (Plasma), Homocystine (Plasma), Homogentisic Acid, Homovanillic Acid, Hydroxykynurenine, Hydroxyproline, Hydroxyproline (Plasma), Indoleacetic Acid, Isocitric Acid, Isoleucine/allo-Isoleucine (Plasma), KT Ratio (Plasma), Kynurenic Acid, Kynurenine (Plasma), Lactic Acid, Leucine (Plasma), Lysine (Plasma), Malic Acid, Mandelic Acid, Methionine (Plasma), Methylmalonic Acid, Microalbumin, Ornithine, Ornithine (Plasma), Orotic Acid, Oxalic Acid, pH, Phenylacetic Acid, Phenylalanine (Plasma), Phosphate, Phosphoethanolamine (Plasma), Picolinic Acid, Pimelic Acid, Proline (Plasma), Pyridoxic Acid, Pyroglutamic Acid, Pyruvic Acid, Quercetin, Quinolinic Acid, Sarcosine (Plasma), Sebacic Acid, Serine (Plasma), Suberic Acid, Suberylglycine, Succinic Acid, Sulfocysteine (Plasma), Tartaric Acid, Taurine (Plasma), Threonine (Plasma), Total Branched Chain Amino Acids (Plasma), Tryptophan (Plasma), Tyrosine (Plasma), Valine (Plasma), Vannilylmandelic Acid, Xanthurenic Acid
