Optimal Result: 0 - 0.49 ug/mg creatinine.

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.

What does it mean if your Alpha-Ketoisovalerate result is too low?

Low levels of alpha-ketoisovalerate may occur if there are low levels of precursors (valine), if there are nutritional enzyme inhibitions, or if a low-activity enzyme variant is inherited. The enzyme that converts valine into alpha-ketoisovalerate is vitamin B6 dependent and this step in the BCAA breakdown pathway is shared by all of the BCAAs. Either a nutritional deficiency or an inherited metabolic disorder may decrease the levels of all the BCAA-derived analytes (alpha-ketoisovalerate, alpha-ketoisocaproate, alpha-keto-beta-methylvalerate, beta-hydroxyisovalerate).

1. Consider supporting synthesis with vitamin B6. If B6 is deficient then alpha-ketoglutarate, fumarate, kynurenate, para-hydroxyphenyllactate, alpha-hydroxybutyrate, para-hydroxyphenylacetate and neurotransmitters may also be lower than expected.

2. BCAA supplementation may have beneficial effects in patients with muscle wasting, which can occur after acute critical illness or with chronic conditions such as cirrhosis, kidney failure, etc.

What does it mean if your Alpha-Ketoisovalerate result is too high?

Higher levels of alpha-ketoisovalerate may occur when there are nutritional enzyme inhibitions of the breakdown pathways, inherited low-activity enzymes are present, if there are high levels of precursors (dietary proteins, amino acids, valine), or if there are higher levels of its downstream products (methylmalonate, methylsuccinate). Higher levels of circulating BCAAs have been associated with obesity, metabolic syndrome, cardiovascular and hypertension risks; valine may be the first to elevate. The risk appears to be associated with low-activity enzymes in the breakdown pathway. Methylmalonate is derived primarily from valine. If alpha-ketoisovalerate is high, there may be a downstream enzyme block that prevents the formation of methylmalonate. Inborn errors of metabolism, such as maple syrup urine disease, increase levels of alpha keto acids which act as neurotoxins in the central nervous system. High levels of alpha-ketoisovalerate may inhibit gluconeogenesis, the urea cycle, and pyruvate metabolism.

1. Consider supporting the breakdown of alpha-ketoisovalerate with vitamins B1, B2, B3, B6, r-lipoic acid, iron (if deficient) and molybdenum.

2. Urinary orotate levels may increase if liver disorders, urea cycle disorders or other inherited metabolic disorders are present. Adipate and suberate may also increase if liver disorders are present. Inborn errors of metabolism may increase the levels of all the keto-acids (alpha-ketoisovalerate, alpha-ketoisocaproate, alpha-keto-beta-methylvalerate, beta-hydroxyisovalerate).

3. Pyruvate and citrate may be low if pyruvate metabolism is inhibited. Check their status in the Glycolysis Metabolites and Citric Acid Cycle Metabolites sections.

4. Methylmalonate is derived primarily from valine. If alpha-ketoisovalerate is high, there may be a downstream block that prevents the formation of methylmalonate, and levels may be lower than expected even if there is a vitamin B-12 deficiency present. The conversion of ethylmalonate to methylsuccinate also requires B12. Look to see if ethylmalonate is high and methylsuccinate is low to double-check B12 status.

5. Evaluate risk of metabolic syndrome or Type II diabetes if indicated.

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