Sorted By Test Name - Mayo Medical Laboratories

AAUCD
60202
Glutamic Acid (Glu)
1-Methylhistidine (1MHis)
3-Methylhistidine (3MHis)
Argininosuccinic Acid (Asa)
Carnosine (Car)
Anserine (Ans)
Homocitrulline (Hcit)
Arginine (Arg) 5-39 11-35 11-27 11-32
Alpha-aminoadipic Acid (Aad)
Gamma-amino-n-butyric Acid
(GABA)
Beta-aminoisobutyric Acid (bAib)
Alpha-amino-n-butyric Acid (Abu)
Hydroxylysine (Hyl)
Proline (Pro)
Ornithine (Orn)
Cystathionine (Cth)
Cystine (Cys)
Lysine (Lys) 11-63 9-33 10-25 13-42
Methionine (Met)
Valine (Val) 14-61 9-28 8-20 11-40
Tyrosine (Tyr) 8-83 5-24 5-17
Homocystine (Hcy)
Isoleucine (Ile)
Leucine (Leu) 12-41 6-21 7-16 7-29
Phenylalanine (Phe) 7-40 5-18 7-21
Tryptophan (Trp)
Allo-isoleucine (AlloIle)
Clinical References: Rinaldo P, Hahn S, Matern D: Inborn errors of amino acid, organic acid, and
fatty acid metabolism. In Tietz Textbook of Clinical Chemistry and Molecular Diagnosis. Fourth edition.
Edited by CA Burtis, ER Ashwood, DE Bruns. St. Louis, WB Saunders Company, 2005, pp 2207-2247
Amino Acids, Urea Cycle Disorders Panel, Plasma
Clinical Information: Urea cycle disorders (UCD) are a group of inherited disorders of amino acid
catabolism that result when any of the enzymes in the urea cycle (carbamoylphosphate synthetase I [CPS
I]; ornithine transcarbamylase [OTC]; argininosuccinic acid synthetase [ASS]; argininosuccinic acid lyase
[ASL]; arginase [ARG]; or the cofactor producer, N-acetyl glutamate synthetase [NAGS]), demonstrate
deficient or reduced activity. The urea cycle serves to break down nitrogen and defects in any of the steps
of the pathway can result in an accumulation of ammonia, which can be toxic to the nervous system.
Infants with a complete enzyme deficiency typically appear normal at birth, but as ammonia levels rise,
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