Skip Ribbon Commands
Skip to main content
Sign In

26299 Acylglycines, Quantitative, Random, Urine (AGU20)

Acylglycines, Quantitative, Random, Urine (AGU20)
Test Code: AGU20SO
Synonyms/Keywords

​2-Methylbutyryl Glycinuria
2-Methylbutyryl-CoA Dehydrogenase Deficiency
EE (Ethylmalonic Encephalopathy)
Ethylmalonic Encephalopathy (EE)
GA 1 (Glutaric Acidemia Type 1)
GA 2 (Glutaric Acidemia Type 2)
GA II (Glutaric Acidemia Type II)
GAII (Glutaric Acidemia Type 2)
GCDH (Glutaryl-CoA Dehydrogenase) Deficiency
Glutaric Acidemia (GA)
Glutaric Acidemia Type 2
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)
MADD
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
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
n-Acetylglycine
n-Propionylglycine
Isobutyrylglycine
Ethylmalonic acid
n-Butyrylglycine
2-Methylsuccinic acid
2-Methylbutyrylglycine
Isovalerylglycine
Glutaric acid
3-Methylcrotonylglycine
n-Tiglylglycine
3-Methylglutaconic acid
n-Hexanoylglycine
n-Octanoylglycine
3-Phenylpropionylglycine
trans-Cinnamoylglycine
Suberylglycine
Dodecanedioic acid
Tetradecanedioic acid
Hexadecanedioic acid

Useful For

Diagnosis and monitoring for patients affected with 1 of the following inborn errors of metabolism:

Fatty Acid Oxidation Disorders

-Glutaric acidemia type II

-Medium-chain 3-ketoacyl-CoA thiolase (MCKAT) deficiency

-Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency

-Short chain acyl-CoA dehydrogenase (SCAD) deficiency

Organic Acidurias

-2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (2M3HBD) deficiency

-2-Methylbutyryl-CoA dehydrogenase deficiency

-3-Methylcrotonyl-CoA carboxylase deficiency

-3-Methylglutaconyl-CoA-hydratase deficiency

-Aminoacylase 1 deficiency

-Beta-ketothiolase deficiency

-Ethylmalonic encephalopathy

-Glutaryl-CoA dehydrogenase deficiency

-Isobutyryl-CoA dehydrogenase (IBD) deficiency

-Isovaleryl-CoA dehydrogenase deficiency

-Multiple carboxylase deficiency

-Propionic acidemia

Highlights:

This test provides a quantitative report of abnormal levels of acylglycines in urine, identified via gas chromatography-mass spectrometry.

Specimen Requirements
Specimen TypePreferred Container/TubeAcceptable Container/TubeSpecimen VolumeSpecimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
​Urine​Plastic, 10-mL urine tube​10 mL​4 mL​If insufficient collection volume, submit as much as possible in a single container; the laboratory will determine if volume is sufficient for testing.
Collection Processing Instructions

​1. Collect a random urine specimen.

2. No preservative.

Specimen Stability Information
Specimen TypeTemperatureTime
​Urine ​​Frozen (preferred)​416 days
​Refrigerated​9 days
Rejection Criteria
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability. 
Performing Laboratory Information
Performing LocationDay(s) Test PerformedAnalytical TimeMethodology/Instrumentation
Mayo Clinic Laboratories​​Monday, Wednesday, Friday​5 days​Gas Chromatography-Mass Spectrometry (GC-MS) Stable Isotope Dilution Analysis
Reference Lab
Reference Range Information
Performing LocationReference Range
​Mayo Clinic Laboratories​See Mayo for reference values based on age and gender. 
Interpretation

​When abnormal results are detected, a detailed interpretation is given including an overview of the results and of their significance; a correlation to available clinical information; elements of differential diagnosis; recommendations for additional biochemical testing and in vitro confirmatory studies (enzyme assay, molecular analysis); name and phone number of key contacts who may provide these studies at Mayo Clinic or elsewhere; and a phone number to reach one of the laboratory directors in case the referring physician has additional questions.

Outreach CPTs
CPTModifier
(if needed)
QuantityDescriptionComments
​82542​1
Synonyms/Keywords

​2-Methylbutyryl Glycinuria
2-Methylbutyryl-CoA Dehydrogenase Deficiency
EE (Ethylmalonic Encephalopathy)
Ethylmalonic Encephalopathy (EE)
GA 1 (Glutaric Acidemia Type 1)
GA 2 (Glutaric Acidemia Type 2)
GA II (Glutaric Acidemia Type II)
GAII (Glutaric Acidemia Type 2)
GCDH (Glutaryl-CoA Dehydrogenase) Deficiency
Glutaric Acidemia (GA)
Glutaric Acidemia Type 2
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)
MADD
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
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
n-Acetylglycine
n-Propionylglycine
Isobutyrylglycine
Ethylmalonic acid
n-Butyrylglycine
2-Methylsuccinic acid
2-Methylbutyrylglycine
Isovalerylglycine
Glutaric acid
3-Methylcrotonylglycine
n-Tiglylglycine
3-Methylglutaconic acid
n-Hexanoylglycine
n-Octanoylglycine
3-Phenylpropionylglycine
trans-Cinnamoylglycine
Suberylglycine
Dodecanedioic acid
Tetradecanedioic acid
Hexadecanedioic acid

Ordering Applications
Ordering ApplicationDescription
​COM​Acylglycines, Qnt, Urine
​Cerner​Acylglycines, Qnt, Ur (AGU20)
​Centricity​Acylglycines, Qnt, Ur (AGU20)
If the ordering application you are looking for is not listed, contact your local laboratory for assistance.
Specimen Requirements
Specimen TypePreferred Container/TubeAcceptable Container/TubeSpecimen VolumeSpecimen Minimum Volume
(allows for 1 repeat)
Pediatric Minimum Volume
(no repeat)
​Urine​Plastic, 10-mL urine tube​10 mL​4 mL​If insufficient collection volume, submit as much as possible in a single container; the laboratory will determine if volume is sufficient for testing.
Collection Processing

​1. Collect a random urine specimen.

2. No preservative.

Specimen Stability Information
Specimen TypeTemperatureTime
​Urine ​​Frozen (preferred)​416 days
​Refrigerated​9 days
Rejection Criteria
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability. 
Useful For

Diagnosis and monitoring for patients affected with 1 of the following inborn errors of metabolism:

Fatty Acid Oxidation Disorders

-Glutaric acidemia type II

-Medium-chain 3-ketoacyl-CoA thiolase (MCKAT) deficiency

-Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency

-Short chain acyl-CoA dehydrogenase (SCAD) deficiency

Organic Acidurias

-2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (2M3HBD) deficiency

-2-Methylbutyryl-CoA dehydrogenase deficiency

-3-Methylcrotonyl-CoA carboxylase deficiency

-3-Methylglutaconyl-CoA-hydratase deficiency

-Aminoacylase 1 deficiency

-Beta-ketothiolase deficiency

-Ethylmalonic encephalopathy

-Glutaryl-CoA dehydrogenase deficiency

-Isobutyryl-CoA dehydrogenase (IBD) deficiency

-Isovaleryl-CoA dehydrogenase deficiency

-Multiple carboxylase deficiency

-Propionic acidemia

Highlights:

This test provides a quantitative report of abnormal levels of acylglycines in urine, identified via gas chromatography-mass spectrometry.

Reference Range Information
Performing LocationReference Range
​Mayo Clinic Laboratories​See Mayo for reference values based on age and gender. 
Interpretation

​When abnormal results are detected, a detailed interpretation is given including an overview of the results and of their significance; a correlation to available clinical information; elements of differential diagnosis; recommendations for additional biochemical testing and in vitro confirmatory studies (enzyme assay, molecular analysis); name and phone number of key contacts who may provide these studies at Mayo Clinic or elsewhere; and a phone number to reach one of the laboratory directors in case the referring physician has additional questions.

For more information visit:
Performing Laboratory Information
Performing LocationDay(s) Test PerformedAnalytical TimeMethodology/Instrumentation
Mayo Clinic Laboratories​​Monday, Wednesday, Friday​5 days​Gas Chromatography-Mass Spectrometry (GC-MS) Stable Isotope Dilution Analysis
Reference Lab
For billing questions, see Contacts
Outreach CPTs
CPTModifier
(if needed)
QuantityDescriptionComments
​82542​1
For most current information refer to the Marshfield Laboratory online reference manual.