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a-Hydroxyisobutyric Acid (from MTBE)

Metabolic Analysis Markers (Urine)

Optimal range:   0 - 6.7 mmol/mol creatinine

a-Hydroxyisobutyric Acid (from MTBE) is a toxic breakdown product.

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a-Keto-b-Methylvalerate

3301 Organix Comprehensive Profile - Urine (mmol/mol creatinine), Genova Diagnostics

Optimal range:   0 - 2.1 mmol/mol creatinine

Alpha-Keto-Beta-Methylvalerate is a B-Complex Vitamin Marker. 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.

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a-Keto-b-methylvaleric Acid

OMX Organic Metabolomics / Diagnostic Solutions, Diagnostic Solutions Laboratory

Optimal range:   0 - 83.5 nmol/mg Creatinine

a-Keto-b-Methylvaleric Acid is a B-Complex Vitamin Marker. 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.

A metabolites of isoleucine.

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a-Keto-b-Methylvaleric Acid

Metabolimix+, Genova Diagnostics

Optimal range:   0 - 2.1 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.

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a-Keto-b-Methylvaleric Acid

Metabolic Analysis Markers (Urine)

Optimal range:   0 - 2.1 mmol/mol creatinine

a-Keto-b-Methylvaleric Acid is a B-Complex Vitamin Marker. 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.

A metabolites of isoleucine.

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a-Keto-b-Methylvaleric Acid

Urine

NutriStat Basic Profile, US BioTek

Optimal range:   0 - 1.52 ug/mgCR

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.

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a-Ketoadipate

3301 Organix Comprehensive Profile - Urine (mmol/mol creatinine), Genova Diagnostics

Optimal range:   0 - 1.7 mmol/mol creatinine

α-Ketoadipic Acid (AKAA; 2-Oxoadipic acid, 2-Ketoadipic acid) is an organic acid formed from α-aminoadipic acid (which originates with lysine) and also from α-aminomuconic acid (derived from tryptophan).

AKAA metabolizes to form glutaryl-CoA via oxidative decarboxylation. The cofactors needed in this step are Coenzyme A, NAD, thiamine pyrophosphate (vitamin B1), lipoic acid, and vitamin B2.

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a-Ketoadipic Acid

Metabolimix+, Genova Diagnostics

Optimal range:   0 - 1.7 mmol/mol creatinine

α-Ketoadipic Acid (AKAA; 2-Oxoadipic acid, 2-Ketoadipic acid) is an organic acid formed from α-aminoadipic acid (which originates with lysine) and also from α-aminomuconic acid (derived from tryptophan).

AKAA metabolizes to form glutaryl-CoA via oxidative decarboxylation. The cofactors needed in this step are Coenzyme A, NAD, thiamine pyrophosphate (vitamin B1), lipoic acid, and vitamin B2.

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a-Ketoadipic Acid

Metabolic Analysis Markers (Urine)

Optimal range:   0 - 1.7 mmol/mol creatinine

Alpha-Ketoadipic acid (or 2-oxoadipate) is an intermediate in the metabolism of lysine.

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a-Ketobutyric Acid

OMX Organic Metabolomics / Diagnostic Solutions, Diagnostic Solutions Laboratory

Optimal range:   0 - 12.6 nmol/mg Creatinine

- Alpha-ketobutyric acid results from the breakdown of threonine or methionine during glutathione production.

- Specifically, cystathionine is metabolized to alpha-ketobutyric acid and cysteine.

- a- ketobutyric acid enters the mitochondrial matrix and get converted to propionyl-CoA by the branched chain keto-acid dehydrogenase complex (BCKDHC) and enters the Krebs cycle at succinyl-CoA.

- Evaluate lactate and the branched chain keto acids

- Evaluate alpha-hydroxybutyric acid

- Associated Nutrients: Vitamin B3

- a -Ketobutyric acid is produced from cystine, along with hydrogen sulfide (H2S) as a by-product.

- a- Ketobutyric acid is reversibly converted to a- hydroxybutyric acid.

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a-Ketoglutarate

3301 Organix Comprehensive Profile - Urine (mmol/mol creatinine), Genova Diagnostics

Optimal range:   4 - 52 mmol/mol creatinine

Alpha-Ketoglutarate is an organic acid that is important for the proper metabolism of all essential amino acids. It is formed in the Krebs cycle, the energy-producing process that occurs in most body cells.

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a-Ketoglutaric Acid

OMX Organic Metabolomics / Diagnostic Solutions, Diagnostic Solutions Laboratory

Optimal range:   0 - 169.6 nmol/mg Creatinine

Alpha-Ketoglutarate is an organic acid that is important for the proper metabolism of all essential amino acids. It is formed in the Krebs cycle, the energy-producing process that occurs in most body cells.

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a-Ketoglutaric Acid

Metabolimix+, Genova Diagnostics

Optimal range:   4 - 52 mmol/mol creatinine

Isocitric Acid is converted to α-ketoglutaric acid using the enzyme isocitrate dehydrogenase. Alphaketoglutarate is a rate-determining intermediate in the Citric Acid Cycle and provides an important source of glutamine and glutamate that stimulates protein synthesis and bone tissue formation, inhibits protein degradation in muscle, and constitutes an important metabolic fuel for cells of the gastrointestinal tract. Alpha-ketoglutaric acid is then converted to Succinyl CoA using the enzyme alpha-ketoglutarate dehydrogenase. This enzyme complex is very similar to the pyruvate dehydrogenase complex with similar nutrient cofactor needs.

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a-Ketoglutaric Acid

Urine

NutriStat Basic Profile, US BioTek

Optimal range:   0 - 21.72 ug/mgCR

Isocitric Acid is converted to α-ketoglutaric acid using the enzyme isocitrate dehydrogenase. Alphaketoglutarate is a rate-determining intermediate in the Citric Acid Cycle and provides an important source of glutamine and glutamate that stimulates protein synthesis and bone tissue formation, inhibits protein degradation in muscle, and constitutes an important metabolic fuel for cells of the gastrointestinal tract. Alpha-ketoglutaric acid is then converted to Succinyl CoA using the enzyme alpha-ketoglutarate dehydrogenase. This enzyme complex is very similar to the pyruvate dehydrogenase complex with similar nutrient cofactor needs.

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a-Ketoglutaric Acid (AKG)

Metabolic Analysis Markers (Urine)

Optimal range:   4 - 52 mmol/mol creatinine

Alpha-Ketoglutarate is an organic acid that is important for the proper metabolism of all essential amino acids. It is formed in the Krebs cycle, the energy-producing process that occurs in most body cells.

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a-Ketoisocaproate

3301 Organix Comprehensive Profile - Urine (mmol/mol creatinine), Genova Diagnostics

Optimal range:   0 - 0.89 mmol/mol creatinine

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

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a-Ketoisocaproic Acid

OMX Organic Metabolomics / Diagnostic Solutions, Diagnostic Solutions Laboratory

Optimal range:   0 - 20.4 nmol/mg Creatinine

2-Ketoisocaproic Acid is a B-Complex Vitamin Marker (Leucine catabolism).

2-Ketoisocaproic Acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids.

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a-Ketoisocaproic Acid

Metabolimix+, Genova Diagnostics

Optimal range:   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.

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a-Ketoisocaproic Acid

Metabolic Analysis Markers (Urine)

Optimal range:   0 - 0.89 mmol/mol creatinine

a-Ketoisocaproic Acid is a B-Complex Vitamin Marker (Leucine catabolism).

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a-Ketoisocaproic Acid

Urine

NutriStat Basic Profile, US BioTek

Optimal range:   0 - 0.34 ug/mgCR

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.

LEARN MORE
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