2-Oxo-4-methiolbutyric acid

Optimal Result: 0 - 0.18 mmol/mol.

2-Oxo-4-methylthiobutanoic acid, also known as 4-(methylsulfanyl)-2-oxobutanoate or 2-keto-4-methylthiobutyrate, belongs to the class of organic compounds known as thia fatty acids. Thia substituted fatty acids are saturated fatty acids which are modified by insertion of a sulfur atom at specific positions in the carbon backbone.

Elevated in an inborn error of methionine metabolism. Confirmation of the genetic disorder requires testing of plasma amino acids.

What does it mean if your 2-Oxo-4-methiolbutyric acid result is too high?

- High 2-Oxo-4-methiolbutyric acid may be due to an error in methionine metabolism.

- Confirmation of the genetic disorder requires testing of plasma amino acids.

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