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3-Methylglutaconic and 3-Methylglutaric Acids - Urine

Referred out for testing

Source Urine
Mnemonic BGLNON

BGL Non-Blood Test Name field: 3-Methylgluta
Specimen Requirements

Prefer 5-10 mL random urine.

Minimum: 1.0 mL urine.

Specimen Handling

If specimen is transferred to secondary container, write "urine" on label. Send a copy of the requisition with the specimen.

PSC:
Send on ice to DSC.

RRL:
Send on ice to ACH.

Extra-regional:
Freeze  before transport to DSC.

DSC:
Freeze specimens upon receipt in lab, ensure frozen specimens do not thaw.

Additional Information

This test is primarily for the investigation of 3-methylglutaconic acidurias, but may also be elevated in a subset of patients with mitochondrial disorders.

This test should not be ordered in conjunction with urine organic acids, as these metabolites are also reported as part of that test, and this test will be reflexively performed on any urine sample with elevated levels detected.

Testing Location ACH Biochemical Genetics Lab
Testing Frequency Monthly; must notify ACH Biochemical Genetics Lab for priority turn-around.
Alternate Name(s) Methylglutaconic Acid - Urine; 3MGC - Urine; Methylglutaric Acid - Urine; 3MGA - Urine
Reference Interval Provided on test report.