Lab Test

Amino Acid Quantitation, CSF

Test Codes

EPIC: LAB7583, Beaker: XAACSF, Mayo: AACSF


Send Outs

Specimen Collection Criteria

Collect: Cerebrospinal Fluid (CSF): Collect specimen from second collection vial.

Note: CRITICAL FROZEN specimen.

Physician Office/Draw Specimen Preparation

Maintain CSF specimen frozen (-20°C/-4°F or below).

Preparation for Courier Transport

Transport: 0.2 mL CSF, frozen (-20°C/-4°F or below). (Minimum: 0.1 mL)

Rejection Criteria

Specimens not collected and processed as indicated.

In-Lab Processing

Maintain CSF specimen frozen (-20°C/-4°F or below).

Transport: 0.2 mL CSF, frozen (-20°C/-4°F or below). (Minimum: 0.1 mL)


Room Temperature (20-26°C or 68-78.8°F): Unacceptable
Refrigerated (2-8°C or 36-46°F): Unacceptable
Frozen (-20°C/-4°F or below): 14 days


Sent to Mayo Clinic Laboratories in Rochester, MN.


Monday – Friday.
Results available in 3-5 days.

Reference Range

By report.

Test Methodology

Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS).

Clinical Utility

Useful for evaluating patients with possible inborn errors of amino acid metabolism, in particular nonketotic hyperglycinemia (glycine encephalopathy) and serine biosynthesis defects, especially when used in conjunction with concomitantly collected plasma specimens.

Testing algorithm 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.

CPT Codes

LOINC:  phosphoserine 26737-7, phosphoethanolamine 26597-5, taurine 26614-8, threonine 22643-1, serine 22644-9, hydroxyproline 26596-7, asparagine 26603-1, glutamic acid 22652-2, 1-methylhistidine 26584-3, 3-methylhistidine 26585-0, argininosuccinic acid 40838-5, carnosine 26591-8, anserine 26588-4, homocitrulline 55875-9, alpha-aminoadipic acid 26587-6, gamma-amino-n-butyric acid 26594-2, beta-aminoisobutyric acid 26590-0, alpha-amino-n-butyric acid 26586-8, hydroxylysine 26595-9, glutamine 22641-5, aspartic acid 22655-5, ethanolamine 26593-4, proline 22645-6,
glycine 22650-6, alanine 22657-1, citrulline 22654-8, sarcosine 26598-3, beta-alanine 26589-2, alpha-amino-n-butyric acid 26586-8, valine 22649-8, cystine 22653-0, methionine 22648-0, isoleucine 22659-7, 30052-5, leucine 9412-8, tyrosine 22642-3, phenylalanine 22646-4, ornithine 22647-2, cystathionine 26592-6, tryptophan 26602-3, allo-isoleucine 22658-9, lysine 22651-4, histidine 9453-2, arginine 22656-3


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