Metabolimix+ (Genova Diagnostics)

a-Ketoisocaproic Acid

Optimal Result: 0 - 0.89 mmol/mol creatinine.

Of the essential amino acids, there are three branchedchain amino acids (leucine, isoleucine, and valine).

Unlike most amino acids, the initial step of branchedchain amino acid (BCAA) metabolism does not take place in the liver. They increase rapidly in systemic circulation after protein intake and are readily available for use. Skeletal muscle is where most of the initial catabolism of BCAA takes place using branched-chain aminotransferase enzymes to form α-ketoacids, which are then released from muscles back into the blood to be further metabolized, mainly in the liver.

BCAA act as substrates for protein synthesis, energy production, neurotransmitter production, glucose metabolism, immune response, and many other beneficial metabolic processes.

α-Ketoisovaleric Acid (AKIV) is produced from the essential amino acid valine. It then metabolizes to become succinyl Co-A. AKIV is glucogenic.

α-Ketoisocaproic Acid (AKIC) is produced from leucine and further metabolizes to form acetyl-CoA and acetoacetate. AKIC is ketogenic.

α-Keto-β-Methylvaleric Acid (AKBM) comes from isoleucine, and further metabolizes to form acetylCoA and succinyl-CoA. AKBM is therefore both glycogenic and ketogenic.

These α-ketoacids then require an enzyme complex called branched-chain α-keto acid dehydrogenase (BCKD) for further metabolism.

This enzyme complex requires multiple vitamin cofactors, such as vitamin B1, B2, B3, B5, and lipoic acid.

References:

- Holecek M. Branched-chain amino acids in health and disease: metabolism, alterations in blood plasma, and as supplements. Nutr Metab. 2018;15(1):33.

- Shibata K, Nakata C, Fukuwatari T. High-performance liquid chromatographic method for profiling 2-oxo acids in urine and its application in evaluating vitamin status in rats. Biosci Biotechnol Biochem. 2016;80(2):304-312.

- Danner DJ, Davidson ED, Elsas LJ. Thiamine increases the specific activity of human liver branched chain α-ketoacid dehydrogenase. Nature. 1975;254(5500):529-530.

- Shibata K, Sakamoto M. Urinary branched-chain 2-oxo acids as a biomarker for function of B-group vitamins in humans. J Nutr Sci Vitaminol. 2016;62(4):220-228.

- Adams SH. Emerging Perspectives on Essential Amino Acid Metabolism in Obesity and the Insulin-Resistant State. Adv Nutr. 2011;2(6):445-456.

- Shibata K, Nakata C, Fukuwatari T. High-performance liquid chromatographic method for profiling 2-oxo acids in urine and its application in evaluating vitamin status in rats. Biosci Biotechnol Biochem. 2016;80(2):304-312.

- Solmonson A, DeBerardinis RJ. Lipoic acid metabolism and mitochondrial redox regulation. J Biol Chem. 2018;293(20):7522- 7530.

What does it mean if your a-Ketoisocaproic Acid result is too high?

Urinary elevations of these ketoacids can be the result of functional need for the vitamin cofactors to support BCKD.

A genetic defect of the α-keto acid dehydrogenase enzyme complex is responsible for maple syrup urine disease, which results in very elevated levels of AKIC, AKIV, AKMB.

Elevated plasma levels of branched-chain amino acids have been associated with insulin resistance as a result of decreased catabolism for energy production. This metabolic disturbance may be compounded by further nutrient deficiencies limiting the activity of the BCKD enzyme.

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