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25683 Amino Acids, Quantitative, CSF (AACSF)

Amino Acids, Quantitative, CSF (AACSF)
Test Code: AACSFSO
Synonyms/Keywords
​NKH (Nonketotic Hyperglycinemia)
Nonketotic Hyperglycinemia (NKH)
Serine Biosynthesis Defects
Test Components
​Includes quantitation of the following amino acids: phosphoserine, phosphoethanolamine, taurine, threonine, serine, hydroxyproline, asparagine, glutamic acid, 1-methylhistidine, 3-methylhistidine, argininosuccinic acid, carnosine, anserine, homocitrulline, alpha-aminoadipic acid, gamma-amino-n-butyric acid, beta-aminoisobutyric acid, alpha-amino-n-butyric acid, hydroxylysine, glutamine, aspartic acid, ethanolamine, proline, glycine, alanine, citrulline, sarcosine, beta-alanine, alpha-amino-n-butyric acid, valine, cystine, methionine, isoleucine, leucine, tyrosine, phenylalanine, ornithine, cystathionine, tryptophan, allo-isoleucine, lysine, histidine, and arginine.
Useful For
​Evaluating patients with possible inborn errors of amino acid metabolism, in particular nonketotic hyperglycinemia and serine biosynthesis defects, especially when used in conjunction with concomitantly drawn plasma specimens.
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 ​CSF ​Sterile vial ​ ​0.2 mL ​0.1 mL ​
Collection Processing Instructions
​ Collect specimen from second collection vial.  Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information. 
Specimen Stability Information
Specimen Type Temperature Time
​CSF ​Frozen ​14 days
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
​Mayo Medical Laboratories ​Monday through Friday 3 days ​LC/MS/MS
Reference Lab
Mayo Clinic Laboratories
Reference Range Information
Performing Location Reference Range
​Mayo Medical Laboratories ​Varies per amino acid.  See Report.
Outreach CPTs
CPT Modifier
(if needed)		 Quantity Description Comments
​82139 ​ ​ ​ ​
Synonyms/Keywords
​NKH (Nonketotic Hyperglycinemia)
Nonketotic Hyperglycinemia (NKH)
Serine Biosynthesis Defects
Test Components
​Includes quantitation of the following amino acids: phosphoserine, phosphoethanolamine, taurine, threonine, serine, hydroxyproline, asparagine, glutamic acid, 1-methylhistidine, 3-methylhistidine, argininosuccinic acid, carnosine, anserine, homocitrulline, alpha-aminoadipic acid, gamma-amino-n-butyric acid, beta-aminoisobutyric acid, alpha-amino-n-butyric acid, hydroxylysine, glutamine, aspartic acid, ethanolamine, proline, glycine, alanine, citrulline, sarcosine, beta-alanine, alpha-amino-n-butyric acid, valine, cystine, methionine, isoleucine, leucine, tyrosine, phenylalanine, ornithine, cystathionine, tryptophan, allo-isoleucine, lysine, histidine, and arginine.
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 ​CSF ​Sterile vial ​ ​0.2 mL ​0.1 mL ​
Collection Processing
​ Collect specimen from second collection vial.  Include family history, clinical condition (asymptomatic or acute episode), diet, and drug therapy information. 
Specimen Stability Information
Specimen Type Temperature Time
​CSF ​Frozen ​14 days
Useful For
​Evaluating patients with possible inborn errors of amino acid metabolism, in particular nonketotic hyperglycinemia and serine biosynthesis defects, especially when used in conjunction with concomitantly drawn plasma specimens.
Test Components
​Includes quantitation of the following amino acids: phosphoserine, phosphoethanolamine, taurine, threonine, serine, hydroxyproline, asparagine, glutamic acid, 1-methylhistidine, 3-methylhistidine, argininosuccinic acid, carnosine, anserine, homocitrulline, alpha-aminoadipic acid, gamma-amino-n-butyric acid, beta-aminoisobutyric acid, alpha-amino-n-butyric acid, hydroxylysine, glutamine, aspartic acid, ethanolamine, proline, glycine, alanine, citrulline, sarcosine, beta-alanine, alpha-amino-n-butyric acid, valine, cystine, methionine, isoleucine, leucine, tyrosine, phenylalanine, ornithine, cystathionine, tryptophan, allo-isoleucine, lysine, histidine, and arginine.
Reference Range Information
Performing Location Reference Range
​Mayo Medical Laboratories ​Varies per amino acid.  See Report.
For more information visit:
Performing Laboratory Information
Performing Location Day(s) Test Performed Analytical Time Methodology/Instrumentation
​Mayo Medical Laboratories ​Monday through Friday 3 days ​LC/MS/MS
Reference Lab
Mayo Clinic Laboratories
For billing questions, see Contacts
Outreach CPTs
CPT Modifier
(if needed)		 Quantity Description Comments
​82139 ​ ​ ​ ​
For most current information refer to the Marshfield Laboratory online reference manual.