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.
- 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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