Ordering Recommendation

Diagnostic evaluation for glutaric acidemia type I. Use in conjunction with urine organic acids and plasma acylcarnitines.




Liquid Chromatography-Tandem Mass Spectrometry




4-11 days

New York DOH Approval Status

Specimens from New York clients will be sent out to a New York DOH approved laboratory, if possible.

Specimen Required

Patient Preparation

Random urine. Avoid dilute urine when possible.

Specimen Preparation

Transfer 2.5 mL urine to an ARUP Standard Transport Tube and freeze immediately. (Min: 1.0 mL)

Storage/Transport Temperature

Frozen. Separate specimens must be submitted when multiple tests are ordered.

Unacceptable Conditions

Specimens that have exposed to more than three freeze/thaw cycles.


Clinical information is needed for appropriate interpretation. Additional required information includes age, gender, diet (e.g., TPN therapy), drug therapy, and family history. Biochemical Genetics Patient History Form is available on the ARUP Web site at https://www.aruplab.com/patienthistory or by contacting ARUP Client Services.


Ambient: Unacceptable; Refrigerated: 1 week; Frozen: 1 month

Reference Interval

Reports include age appropriate reference interval.

Available Separately
Reference Interval
No Glutarylcarnitine, Urine Less than or equal to 2.0 mmol/mol creatinine

Interpretive Data

Methodology: Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)

If patient is receiving carnitine supplements, results may not be informative.  Clinical correlation is recommended for interpretation of the result.

This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.

Compliance Category

Laboratory Developed Test (LDT)


Hotline History


CPT Codes



Component Test Code* Component Chart Name LOINC
2001511 Glutarylcarnitine, Urine 54279-5
2001513 Creatinine, Urine 2161-8
2002778 Glutarylcarnitine, Urine Interpretation 48767-8
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.


  • Acylcarnitines, urine
  • C5-DC Acylcarnitine
  • GA 1
  • GA-1
  • GA1
  • GCDH Def
  • Glutaric Aciduria 1
  • Glutaric Aciduria Type I
  • Glutaryl CoA Dehydrogenase Deficiency
Glutarylcarnitine Quantitative, Urine