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Denver Genetic Laboratories

D- and L- Isomers of 2-Hydroxyglutaric Acid, Qualitative, Urine

Clinical Test Information - CHCO Biochemical Genetics Laboratory


CPT Code:



Monday - Friday, 0800 - 1700.

Turnaround Time:

Three weeks.

Minimum Volume:

5.0 mL.

Specimen Handling:

Random urine, frozen without preservative.

Interpretive Reference Range:

Subjective interpretation of organic acid screen; n/a to isomer type.


To determine isomeric form of increased 2-hydroxyglutaric acid.


Gas chromatography/mass spectrometry is used in a two-stage test:

  1. An organic acid screen is run to confirm an increase in 2-hydroxyglutaric acid.
  2. Chiral derivatives of the D- and L- isomers are separated by gas chromatography and identified by mass spectrometry.