Optimal Result: 0 - 1.09 ug/mg creatinine.

Alpha-Ketoisocaproate is a B-Complex Vitamin Marker (Leucine catabolism).

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.

Many B vitamins are used as co-factors in the breakdown of amino acids. A deficiency of any of these B vitamins may lead to a block in one or more of these pathways resulting in elevations of the markers.
Vitamins B1 (thiamin), B3 (niacin), and B5 (pantothenic acid) are necessary for energy pathways of all of the cells in your body. As your food is broken down, specific compounds are formed at steps that require B vitamin assistance. Alpha-Ketoisocaproate is one some these compounds. The production of this metabolite requires vitamins B1, B3, B5 and lipoic acid.

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

Low levels of alpha-ketoisocaproate may occur if there are low levels of precursors (leucine), if there are nutritional enzyme inhibitions, or if a low-activity enzyme variant is inherited. 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).

- 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.

- 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-Ketoisocaproate result is too high?

High levels of alpha-ketoisocaproate 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 (protein, amino acids, leucine), or if there are higher levels of its downstream products. Higher levels of circulating BCAAs have been associated with obesity, metabolic syndrome, cardiovascular and hypertension risks. Higher circulating levels of leucine and isoleucine have been associated with cardiovascular risk. The risk appears to be associated with low-activity enzymes in the breakdown pathway. Patients with liver disorders may not be able to convert alpha-ketoisocaproate back into leucine, which can also increase levels. 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.

1. Consider supporting the breakdown of alpha-ketoisocaproate with vitamins B1, B2, B3, B6, and r-lipoic acid. If beta-hydroxyisovalerate is also high, consider biotin.

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. Evaluate risk of metabolic syndrome or type II diabetes if indicated.

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