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25680 Acylglycines, Quantitative, Urine (ACYLG)

Acylglycines, Quantitative, Urine (ACYLG)
Test Code: ACYLGSO
Synonyms/Keywords
2-Methylbutyryl-CoA Dehydrogenase Deficiency
EE (Ethylmalonic Encephalopathy)
Ethylmalonic Encephalopathy (EE)
GA II (Glutaric Acidemia Type II)
GCDH (Glutaryl-CoA Dehydrogenase) Deficiency
Glutaric Acidemia (GA)
Glutaric Acidemia Type I (GA I)
Glutaric Acidemia Type II (GA II)
Glutaryl-CoA Dehydrogenase (GCDH) Deficiency
Isovaleric Acidemia (IVA)
Isovaleryl-CoA Dehydrogenase (IVD)
IVA (Isovaleric Acidemia)
MCAD (Medium-Chain Acyl-CoA Dehydrogenase) Deficiency
MCKAT (Medium-Chain 3-Ketoacyl-CoA Thiolase) Deficiency
Medium-Chain 3-Ketoacyl-CoA Thiolase (MCKAT) Deficiency
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency
Methylmalonic Acidemia
Methylmalonic Aciduria (MMA)
MMA (Methylmalonic Aciduria)
SBCAD (Short/Branched-Chain Acyl-CoA Dehydrogenase) Deficiency
SCAD (Short-Chain Acyl-CoA Dehydrogenase) Deficiency
Short-Chain Acyl-CoA Dehydrogenase (SCAD) Deficiency
Short/Branched-Chain Acyl-CoA Dehydrogenase (SBCAD) Deficiency
2-Methylbutyryl Glycinuria
GAII (Glutaric Acidemia Type 2)
GA 1 (Glutaric Acidemia Type 1)
Glutaric Acidemia Type 2 (order OAU and ACYLG)
MADD (order OAU and ACYLG)
GA 2 (Glutaric Acidemia Type 2)
Test Components

Ethylmalonic Acid
2-Methylsuccinic Acid
Glutaric Acid
Isobutyrylglycine 
n-Butyrylglycine 
2-Methylbutyrylglycine 
Isovalerylglycine 
n-Hexanoylglycine 
n-Octanoylglycine 
3-Phenylpropionylglycine 
Suberylglycine 
trans-Cinnamoylglycine 
Dodecanedioic Acid (12 DCA) 
Tetradecanedioic Acid (14 DCA) 
Hexadecanedioic Acid (16 DCA)

Useful For
​Newborn screening follow-up
Specimen Requirements
Fasting Required Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)		 Pediatric Minimum Volume
(no repeat)
​No ​Random Urine (no preservative) ​Plastic tube ​ ​10 mL ​4 mL ​
Collection Processing Instructions
If insufficient collection volume, submit as much specimen as possible in a single container; the laboratory will determine if volume is sufficient for testing. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.
Specimen Stability Information
Specimen Type Temperature Time
​Urine ​ ​Frozen (preferred) ​416 days
​Refrigerate ​9 days
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
​Mayo Medical Laboratories ​Monday, Wednesday, Friday ​7 days ​Capillary gas chromatography/mass spectrometry
Reference Lab
Mayo Clinic Laboratories
Reference Range Information
Performing Location Reference Range
​Mayo Medical Laboratories ​Varies per component.  See Report.
Outreach CPTs
CPT Modifier
(if needed)		 Quantity Description Comments
​82542 ​ ​ ​ ​
Synonyms/Keywords
2-Methylbutyryl-CoA Dehydrogenase Deficiency
EE (Ethylmalonic Encephalopathy)
Ethylmalonic Encephalopathy (EE)
GA II (Glutaric Acidemia Type II)
GCDH (Glutaryl-CoA Dehydrogenase) Deficiency
Glutaric Acidemia (GA)
Glutaric Acidemia Type I (GA I)
Glutaric Acidemia Type II (GA II)
Glutaryl-CoA Dehydrogenase (GCDH) Deficiency
Isovaleric Acidemia (IVA)
Isovaleryl-CoA Dehydrogenase (IVD)
IVA (Isovaleric Acidemia)
MCAD (Medium-Chain Acyl-CoA Dehydrogenase) Deficiency
MCKAT (Medium-Chain 3-Ketoacyl-CoA Thiolase) Deficiency
Medium-Chain 3-Ketoacyl-CoA Thiolase (MCKAT) Deficiency
Medium-Chain Acyl-CoA Dehydrogenase (MCAD) Deficiency
Methylmalonic Acidemia
Methylmalonic Aciduria (MMA)
MMA (Methylmalonic Aciduria)
SBCAD (Short/Branched-Chain Acyl-CoA Dehydrogenase) Deficiency
SCAD (Short-Chain Acyl-CoA Dehydrogenase) Deficiency
Short-Chain Acyl-CoA Dehydrogenase (SCAD) Deficiency
Short/Branched-Chain Acyl-CoA Dehydrogenase (SBCAD) Deficiency
2-Methylbutyryl Glycinuria
GAII (Glutaric Acidemia Type 2)
GA 1 (Glutaric Acidemia Type 1)
Glutaric Acidemia Type 2 (order OAU and ACYLG)
MADD (order OAU and ACYLG)
GA 2 (Glutaric Acidemia Type 2)
Test Components

Ethylmalonic Acid
2-Methylsuccinic Acid
Glutaric Acid
Isobutyrylglycine 
n-Butyrylglycine 
2-Methylbutyrylglycine 
Isovalerylglycine 
n-Hexanoylglycine 
n-Octanoylglycine 
3-Phenylpropionylglycine 
Suberylglycine 
trans-Cinnamoylglycine 
Dodecanedioic Acid (12 DCA) 
Tetradecanedioic Acid (14 DCA) 
Hexadecanedioic Acid (16 DCA)

Ordering Applications
Ordering Application Description
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Fasting Required Specimen Type Preferred Container/Tube Acceptable Container/Tube Specimen Volume Specimen Minimum Volume
(allows for 1 repeat)		 Pediatric Minimum Volume
(no repeat)
​No ​Random Urine (no preservative) ​Plastic tube ​ ​10 mL ​4 mL ​
Collection Processing
If insufficient collection volume, submit as much specimen as possible in a single container; the laboratory will determine if volume is sufficient for testing. Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information.
Specimen Stability Information
Specimen Type Temperature Time
​Urine ​ ​Frozen (preferred) ​416 days
​Refrigerate ​9 days
Useful For
​Newborn screening follow-up
Test Components

Ethylmalonic Acid
2-Methylsuccinic Acid
Glutaric Acid
Isobutyrylglycine 
n-Butyrylglycine 
2-Methylbutyrylglycine 
Isovalerylglycine 
n-Hexanoylglycine 
n-Octanoylglycine 
3-Phenylpropionylglycine 
Suberylglycine 
trans-Cinnamoylglycine 
Dodecanedioic Acid (12 DCA) 
Tetradecanedioic Acid (14 DCA) 
Hexadecanedioic Acid (16 DCA)

Reference Range Information
Performing Location Reference Range
​Mayo Medical Laboratories ​Varies per component.  See Report.
For more information visit:
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
​Mayo Medical Laboratories ​Monday, Wednesday, Friday ​7 days ​Capillary gas chromatography/mass spectrometry
Reference Lab
Mayo Clinic Laboratories
For billing questions, see Contacts
Outreach CPTs
CPT Modifier
(if needed)		 Quantity Description Comments
​82542 ​ ​ ​ ​
For most current information refer to the Marshfield Laboratory online reference manual.