Glutarylcarnitine Quantitative, Urine
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
This test is New York DOH approved.
ARUP Consult®
Disease Topics
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: 0.5 mL) 
Storage/Transport Temperature
CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered
Unacceptable Conditions
Specimens that have exposed to more than one freeze/thaw cycle. 
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 or by contacting ARUP Client Services. 
Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month 
Reference Interval
Reports include age appropriate reference interval.
Available Separately
Reference Interval
NoGlutarylcarnitine, Urine0.0 - 0.5 mmol/mol creatinine

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

Compliance Statement B: For laboratory developed tests not using a RUO kit, and for FDA approved, cleared or 510(k) exempt assays with alterations. This test was developed and its performance characteristics determined by ARUP Laboratories. The U. S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions.

Hotline History
Component Test Code*Component Chart NameLOINC
2001511Glutarylcarnitine, Urine54279-5
2002778Glutarylcarnitine, Urine Interpretation48767-8
* 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