Amino Acids Quantitative by LC-MS/MS, CSF
Ordering Recommendation

Aid in the diagnosis of selected neurotransmitter disorders (eg, glycine encephalopathy, brain serine deficiency).

Quantitative Liquid Chromatography/Tandem Mass Spectrometry
2-5 days
New York DOH Approval Status
This test is New York DOH approved.
ARUP Consult®
Disease Topics
Specimen Required
Patient Preparation
Specimen Preparation
Centrifuge CSF to separate and remove cellular material. Freeze supernatant. Transport 0.5 mL CSF. (Min: 0.3 mL) 
Storage/Transport Temperature
CRITICAL FROZEN. Separate specimens must be submitted when multiple tests are ordered. 
Unacceptable Conditions
Clinical information is needed for appropriate interpretation. Additional required information includes age, gender, diet (e.g., TPN therapy), drug therapy, and family history. Biochemical Genetics Patient History Form is available on the ARUP Web site at or by contacting ARUP Client Services. 
Ambient: Unacceptable; Refrigerated: 24 hours; Frozen: 1 month 
Reference Interval
Effective May 18, 2015
ComponentsReference IntervalComponentsReference Interval
Alpha-Aminoadipic acid0.0 - 2.0 µmol/LHistidine5.0 - 24.0 µmol/L
Alpha-Amino-n-butyric acid0.0 - 6.5 µmol/LHomocitrulline0.0 - 4.0 µmol/L
Alanine12.5 - 47.3 µmol/LHomocystine0.0 - 2.0 µmol/L
Alloisoleucine0.0 - 2.0 µmol/LHydroxylysine0.0 - 5.0 µmol/L
Anserine0.0 - 7.0 µmol/LHydroxyproline0.0 - 8.0 µmol/L
Arginine5.9 - 30.6 µmol/LIsoleucine1.0 - 11.0 µmol/L
Argininosuccinic acid0.0 - 2.0 µmol/LLeucine3.4 - 25.9 µmol/L
Asparagine0.0 - 23.6 µmol/LLysine7.8 - 40.8 µmol/L
Aspartic acid0.0 - 5.8 µmol/LMethionine0.0 - 10.0 µmol/L
Beta-Alanine2.7 - 21.7 µmol/LOrnithine2.0 - 12.0 µmol/L
Beta-Aminoisobutyric acid0.0 - 2.0 µmol/LPhenylalanine6.9 - 25.1 µmol/L
Citrulline0.0 - 5.6 µmol/LProline0.0 - 8.0 µmol/L
Cystathionine0.0 - 3.0 µmol/LSarcosine0.0 - 2.0 µmol/L
Cystine0.0 - 5.0 µmol/LSerine18.0 - 73.0 µmol/L
Ethanolamine3.8 - 29.0 µmol/LTaurine2.7 - 16.2 µmol/L
Gamma-Amino-n-butyric acid0.0 - 2.0 µmol/LThreonine10.0 - 60.0 µmol/L
Glutamic acid0.0 - 15.0 µmol/LTryptophan0.0 - 6.0 µmol/L
Glutamine230.7 - 637.4 µmol/LTyrosine5.4 - 23.7 µmol/L
Glycine3.1 - 21.0 µmol/LValine7.0 - 37.1 µmol/L

Interpretive Data

Compliance Statement B: For laboratory developed tests not using a RUO kit, and for FDA approved, cleared or 510(k) exempt assays with alterations. This test was developed and its performance characteristics determined by ARUP Laboratories. The U. S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions.

Hotline History
Component Test Code*Component Chart NameLOINC
0080138Alanine, CSF22657-1
0080139Arginine, CSF22656-3
0080141Asparagine, CSF26603-1
0080142Aspartic Acid, CSF22655-5
0080143Citrulline, CSF22654-8
0080144Cystine, CSF22653-0
0080148Glutamine, CSF22641-5
0080149Glutamic Acid, CSF22652-2
0080151Glycine, CSF22650-6
0080152Histidine, CSF9453-2
0080153Homocystine, CSF22660-5
0080154Hydroxyproline, CSF26596-7
0080156Isoleucine, CSF22659-7
0080157Leucine, CSF9412-8
0080158Lysine, CSF22651-4
0080159Methionine, CSF22648-0
0080161Ornithine, CSF22647-2
0080162Phenylalanine, CSF22646-4
0080163Proline, CSF22645-6
0080167Serine, CSF22644-9
0080168Taurine, CSF26614-8
0080169Threonine, CSF22643-1
0080171Tyrosine, CSF22642-3
0080172Valine, CSF22649-8
0080173Amino Acids, CSF Interpretation48767-8
2011976Homocitrulline, CSF55875-9
2011977Hydroxylysine, CSF26595-9
2011983Sarcosine, CSF26598-3
2011984Tryptophan, CSF26602-3
2011985Alpha-amino butyric acid, CSF26586-8
2011986Allo-isoleucine, CSF22658-9
2011987Alpha-aminoadipic acid, CSF26587-6
2011988Anserine, CSF26588-4
2011989Argininosuccinic Acid, CSF40838-5
2011990Beta-alanine, CSF26589-2
2011991Beta-amino isobutyric acid, CSF26590-0
2011992Cystathionine, CSF26592-6
2011993Ethanolamine, CSF26593-4
2011994Gamma-amino butyric acid, CSF26594-2
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please click the sidebar link to access the Interface Map.
  • CSF amino acids
  • quantitative analysis of amino acids