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argininosuccinic acid

   Also found in: Wikipedia 0.01 sec.
argininosuccinic acid /ar·gi·ni·no·suc·cin·ic ac·id/ (ASA) (-suk-sin´ik) an amino acid formed in the urea cycle.
ar·gi·ni·no·suc·cin·ic acid (ärj-n-n-sk-snk, ärj-n-)
n.
An acid formed as an intermediate during the urea cycle in a reaction involving aspartic acid and adenosine triphosphate.
argininosuccinic acid [ahr″-jĭ-ne´no-suk-sin″ik]
a compound normally formed in urea formation in the liver, but not normally present in urine.
argininosuccinic acid
a compound normally formed as an intermediate in urea metabolism in the liver, but not normally present in urine.
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MS/MS has proven to be ineffective in reliably identifying deficiencies in the urea-cycle enzymes ornithine transcarbamylase (OTC) and carbamyl phosphate synthetase (CPSI) through the quantitation of citrulline, though is considerably more useful when detecting elevations in this analyte in newborns with a deficiency in argininosuccinic acid synthetase (ASAS) or argininosuccinic acid lyase (ASAL).
Potential and pitfalls of NBS, and the reference lab's role by Bartholomew, Dennis William / Medical Laboratory Observer
Citrullinemia--Citrullinemia stems from a deficiency of argininosuccinic acid synthetase.
Tandem Mass Spectrometry by Roe, Charles R.; Sweetman, Lawrence / The Exceptional Parent
About HPN-100 (glycerol phenylbutyrate) HPN-100 (glycerol phenylbutyrate), an investigational product, is a pre-pro-drug of phenylacetic acid, the active moiety of BUPHENYL([R]), the only therapy currently FDA-approved as adjunctive therapy for the chronic management of patients with the most prevalent urea cycle disorders: carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), and argininosuccinic acid synthetase (AS) deficiencies.
Hyperion Therapeutics Announces First Patient Enrolled in Pivotal ... by Business Wire
 
 
 
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