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
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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2-Hydroxyphenylacetic Acid, 3-Hydroxyisovaleric Acid, 3-Hydroxyphenylacetic Acid, 3-Hydroxypropionic Acid, 3-Methyl-4-OH-phenylglycol, 4-Hydroxyphenylacetic Acid, 5-OH-indoleacetic Acid, a-Hydroxyisobutyric Acid (from MTBE), a-Keto-b-Methylvaleric Acid, a-Ketoadipic Acid, a-Ketoglutaric Acid (AKG), a-Ketoisocaproic Acid, a-Ketoisovaleric Acid, a-Ketophenylacetic Acid (from Styrene), Adipic Acid, Arabinose, B-OH-B-Methylglutaric Acid (HMG), Benzoic Acid, Beta-OH-Butyric Acid (BHBA), Cis-Aconitic Acid, Citramalic Acid, Citric Acid, Dihydroxyphenylpropionic Acid (DHPPA), Formiminoglutamic Acid (FIGlu), Glutaric Acid, Hippuric Acid, Homogentisic Acid, Homovanillic Acid, Indoleacetic Acid (IAA), Isocitric Acid, Isovalerylglycine, Kynurenic / Quinolinic Ratio, Kynurenic Acid, Lactic Acid, Malic Acid, Methylmalonic Acid, Orotic Acid, Phenylacetic Acid (PAA), Pyroglutamic Acid, Pyruvic Acid, Quinolinic Acid, Suberic Acid, Succinic Acid, Tartaric Acid, Vanilmandelic Acid, Xanthurenic Acid
