Alpha-Ketoisovalerate (together with Alpha-Ketoisocaproate and Alpha-Keto-Beta-methylvalerate) requires Vitamins B1, B2, B3, B5 and lipoic acid to be metabolized.
Nutrient associations: Vitamins B1, B2, B3, B5
What do elevations mean?
Elevations of these organic acids can indicate insufficiency of these B vitamins, especially B1 and B5.
Why are these B vitamins so critical?
These B vitamins are involved in neurological function and are critical for childhood development and learning. A variety of disorders have been associated with lack of these vitamins, including memory loss, anemia, and dermatitis.
Vitamins B1 (thiamin), B3 (niacin), and B5 (pantothenic acid) are necessary for energy pathways of all of the cells in your body. As food is broken down in your body, specific compounds are formed (such as Alpha-Ketoisovalerate) at steps that require B vitamin assistance.
Possible low B6; OR secondary to low branched-chain amino acids.
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Impaired metabolism due to cofactor insufficiencies or toxic metals; OR 'maple syrup urine disease' (if markedly elevated).
Treatment options:
Supplement co-factors (vitamins B1, B2, B3, B5, Mg, cysteine, or lipoic acid); remove toxic metals (As, Hg, Sb, Cd).
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2-Methylhippurate, 3,4-Dihydroxyphenylpropionate, 5-Hydroxyindoleacetate, 8-Hydroxy-2-deoxyguanosine, Adipate, Alpha-Hydroxybutyrate, Alpha-Keto-Beta-Methylvalerate, Alpha-Ketoglutarate, Alpha-Ketoisocaproate, Alpha-Ketoisovalerate, Benzoate, Beta-Hydroxybutyrate, Beta-Hydroxyisovalerate, cis-Aconitate, Citrate, D-Arabinitol, D-Lactate, Ethylmalonate, Formiminoglutamate, Fumarate, Glucarate, Hippurate, Histamine, Homovanillate (Genova), Hydroxymethylglutarate, Indican, Isocitrate, Ketoisovalerate, Kynurenate, L-Lactate, Malate, Methylmalonate (Genova), Orotate, p-Hydroxybenzoate, p-Hydroxyphenylacetate, p-Hydroxyphenyllactate, Phenylacetate, Phenylpropionate, Picolinate, Pyroglutamate (Genova), Pyruvate (Genova), Quinolinic acid (Quinolinate) (Genova), Suberate, Succinate, Sulfate, Tricarballylate, Vanilmandelate (Genova), Xanthurenate (Genova)
