What is Ketoisovalerate? High and low values | Lab results explained

AKA: Alpha-Ketoisovalerate

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Alpha-Ketoisovalerate requires Vitamin B1, B5, lipoic acid, B2, and B3 (in descending order of significance) to be metabolized. As your food is broken down, specific compounds are formed at steps that require B vitamin assistance. a-Ketoisovalerate is one of these compounds. If these nutrients are insufficient, the keto acids may build up in the urine.

If pyruvate and α-ketoglutarate are also both elevated, then there may be a strong need for these specific nutrients since all of them utilize a dehydrogenase enzyme.

Higher levels:

– Elevations can indicate insufficiency of B vitamins, especially B1 and B5. 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.
– Impaired metabolism due to cofactor insufficiencies or toxic metals; OR “maple syrup urine disease” (if markedly elevated).
– Supplement cofactors (vitamins B1, B2, B3, B5, Mg, cysteine, or lipoic acid); remove toxic metals (As, Hg, Sb, Cd)

Lower levels:

– Possible low B6; OR secondary to low branched-chain amino acids.
– If you are on Clofibrate (Clofibrate is a lipid lowering agent used for controlling the high cholesterol) this marker might have diminished sensitivity.


The information on is NOT intended to replace a one-on-one relationship with a qualified health care professional and is not intended as medical advice.


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