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Optimal range: 0 - 1005 mcg/g
2-Hydroxyisobutyric acid is formed endogenously as a product of branched-chain amino acid degradation and ketogenesis. This compound is also the major metabolite of gasoline octane enhancers such as MTBE and ETBE.
Optimal range: 0 - 200 µg/g creatinine
2-Hydroxyisobutyric acid is formed endogenously as a product of branched-chain amino acid degradation and ketogenesis. This compound is also the major metabolite of gasoline octane enhancers such as MTBE and ETBE.
Optimal range: 0 - 795 ug/g
2-Hydroxyisobutyric acid is formed endogenously as a product of branched-chain amino acid degradation and ketogenesis. This compound is also the major metabolite of gasoline octane enhancers such as MTBE and ETBE.
MTBE and ETBE are gasoline additives used to improve octane ratings. Exposure to these compounds is most likely due to groundwater contamination, inhalation or skin exposure to gasoline or its vapors, and exhaust fumes. MTBE has been demonstrated to cause hepatic, kidney, and central nervous system toxicity, peripheral neurotoxicity, and cancer in animals. Very high values have been reported in genetic disorders. Because the metabolites of these compounds are the same, ETBE may be similarly toxic.
Optimal range: 0 - 2 mmol/mol creatinine
2-Hydroxyisocaproic acid (aka Leucic acid / α-hydroxyisocaproic acid / HICA) is a metabolite of the branched-chain amino acid leucine.
Optimal range: 0 - 0 mmol/mol creatinine
2-Hydroxyisocaproic acid (aka Leucic acid / α-hydroxyisocaproic acid / HICA) is a metabolite of the branched-chain amino acid leucine.
Optimal range: 0 - 2 mmol/mol creatinine
2-Hydroxyisocaproic acid (aka Leucic acid / α-hydroxyisocaproic acid / HICA) is a metabolite of the branched-chain amino acid leucine.
Optimal range: 0 - 2 mmol/mol creatinine
LEARN MOREOptimal range: 0 - 0.88 mmol/mol
2-Hydroxyisocaproic acid (aka Leucic acid / α-hydroxyisocaproic acid / HICA) is a metabolite of the branched-chain amino acid leucine.
Optimal range: 0 - 1 mmol/mol creatinine
2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite.
Optimal range: 0 - 2 mmol/mol creatinine
2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite.
Optimal range: 0 - 2 mmol/mol creatinine
2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite.
Optimal range: 0 - 2 mmol/mol creatinine
2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite.
Optimal range: 0 - 0.4 mmol/mol
2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite.
2-hydroxy-3-methylbutyric acid is a valine derivative that is valine in which the amino group has been replaced by a ‘hydroxy’ group.
A moderate increase of branched-chain amino acid metabolites in urine may result from lactic acidosis, episodic ketosis, or de ficiencies of the vitamins, thiamine or lipoic acid. A signi ficant increase of branched-chain amino acid metabolites is associated with the genetic disorders, maple syrup urine disease (MSUD) and pyruvate dehydrogenase de ficiency. Patients with slight to moderate elevations may use dietary supplements containing thiamine to improve clinical symptoms.
Optimal range: 0 - 1.24 ug/mg creatinine
2-Hydroxyphenylacetate is a key intermediary in the metabolism of phenylalanine, an essential amino acid, and tyrosine, a conditionally essential amino acid. Elevated levels of 2-Hydroxyphenylacetate can signal underlying metabolic disturbances or genetic abnormalities in phenylalanine and tyrosine metabolism, such as phenylketonuria (PKU) or tyrosinemia. Monitoring 2-Hydroxyphenylacetate levels as part of the Organic Acids Profile can be instrumental in diagnosing and managing these metabolic disorders, facilitating early intervention and personalized treatment strategies for affected individuals.
Optimal range: 0.06 - 0.66 mmol/mol creatinine
The metabolite of phenylalanine via phenyl pyruvate.
Optimal range: 0.03 - 0.47 mmol/mol creatinine
The metabolite of phenylalanine via phenyl pyruvate.
Optimal range: 0 - 0.86 mmol/mol creatinine
The metabolite of phenylalanine via phenyl pyruvate.
Optimal range: 0.05 - 0.69 mmol/mol
The metabolite of phenylalanine via phenyl pyruvate.
Optimal range: 0 - 0.76 mmol/mol creatinine
Metabolite of phenylalanine via phenyl pyruvate.
Optimal range: 10 - 30 umol/L
2-Hydroxy-3-methylpentanoic acid or 2-hydroxy-3-methylvaleric acid (HMVA) is an organic acid generated by L-isoleucine metabolism. It is derived from the reduction of 2-Keto-3-methylvaleric acid (KMVA), possibly through the action of a lactate dehydrogenase.
