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Acylglycine Profile

Item Value
Approval req'd? No
Available Stat? No
Test code AGLY
Performed by Mayo
Sendout? Yes
Method Stable Isotope Dilution/GCMS
Container type Urine cup
Amount to Collect 20 mL urine
Sample type Random urine
Preferred volume 5 mL urine
Min. Volume 3 mL urine
Processing notes Freeze at -20C. Order MAYO# 81249 PT54490. Forward specimen frozen to China Basin Sendout for Mayo courier pickup Monday-Friday at 1600 hours.
Units µg/mg creatinine
Normal range
2-Ethylmalonic Acid 0.5-20.2 µg/mg creatinine
2-Methylsuccinic Acid 0.4-13.8 µg/mg creatinine
Glutaric Acid 0.6-15.2 µg/mg creatinine
Isobutyrylglycine < 0.01-11.0 µg/mg creatinine
n-Butyrylglycine 0.1-2.1 µg/mg creatinine
2-Methylbutyrylglycine 0.3-7.5 µg/mg creatinine
Isovalerylglycine 0.3-14.3 µg/mg creatinine
n-Hexanoylglycine 0.2-1.9 µg/mg creatinine
n-Octanoylglycine 0.1-2.1 µg/mg creatinine
3-Phenylpropionylglycine < 0.01-1.1 µg/mg creatinine
Suberylglycine < 0.01-11.0 µg/mg creatinine
trans-Cinnamoylglycine 0.2-14.7 µg/mg creatinine
Dodecanedioic Acid (12 DCA) 0.1-1.1 µg/mg creatinine
Tetradodecanedioic Acid (14 DCA) < 0.01-1.0 µg/mg creatinine
Hexadodecanedioic Acid (16 DCA) < 0.01-1.0 µg/mg creatinine
Synonyms Acyl-glycine; MCAD; Medium chain acyl-coenzyme a dehydrogenase deficiency; Medium chain fatty acid oxidation defect; Hexanoylglycine; Phenylpropionylglycine; Suberylglycine
Turn around times Test performed weekly. Turnaround time: 2-9 days.
Additional information For possible fatty acid oxidation abnormalities due to a mutation of acylcoenzyme A dehydrogenase but undetected by urinary organic acid analysis. Elevation of hexanoyl- and phenylpropionylglycine is characteristic of deficient activity of medium-chain acylcoenzyme A dehydrogenase.
CPT coding 82544-90
LOINC code 13753-9
Last Updated 3/24/2013 7:18:05 PM
Entry Number 13

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