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3-Methylglutaconic and 3-Methylglutaric Acids - Blood

Referred out for testing

Source Blood
Mnemonic BGLBL

BGL Test Name field: 3-Methylgluta
Specimen Requirements

1 x 4 mL dark green top sodium heparin tube. 1 x 4 mL dark green lithium heparin, NOT PST, and red top non-additive tube are also acceptable.

Minimum 0.75 mL plasma or serum.

Requires a minimum four-hour fast, or just prior to next feed for newborns.

Specimen Handling

Send a copy of the requisition with the specimen.

PSC:
Centrifuge, transfer plasma or serum to aliquot tube, write "NaHep", "LiHep"  or "Red Top" on the aliquot tube, and transport on ice to DSC.

RRL:
Centrifuge, transfer plasma or serum to aliquot tube, write "NaHep", "LiHep" or "Red Top" on the aliquot tube, and transport on ice to ACH.

Extra-regional:
Centrifuge, transfer plasma or serum to aliquot tube, write "NaHep", "LiHep" or "Red Top" on the aliquot tube, and freeze prior to transport to DSC.

DSC:
Freeze plasma or serum 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.

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