argininosuccinic acid

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Related to argininosuccinic acid: Argininosuccinic aciduria

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.

ar·gi·ni·no·suc·cin·ic ac·id

(ar'ji-ni-nō-sŭk-sin'ik as'id),

Compound formed as an intermediate in the conversion of l-citrulline to l-arginine in the urea cycle.

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 ac·id

(ahr'ji-ni-nō-sŭk-sin'ik as'id)

Formed as an intermediate in the conversion of l-citrulline to l-arginine in the urea cycle.

argininosuccinic acid

a compound normally formed as an intermediate in urea metabolism in the liver, but not normally present in urine.

References in periodicals archive ?

HPN-100 is a pre-pro-drug of phenylacetic acid which is 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 deficiencies including those related to carbamylphosphate synthetase, ornithine transcarbamylase and argininosuccinic acid synthetase.

Hyperion Therapeutics Receives Orphan Drug Designation for HPN-100 for the Treatment of Urea Cycle Disorders

A separate solution of glutamine and argininosuccinic acid (Sigma-Aldrich) in water, stored at -20[degrees]C, was also used.

Year-long validation study and reference values for urinary amino acids using a reversed-phase HPLC method

HPN-100 (glycerol phenylbutyrate), an investigational product, is a pre-pro-drug of phenylacetic acid, the active moiety of BUPHENYL([R]) (sodium phenylbutyrate) Tablets and Powder, 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 Enrolls First Patient in Phase II Hepatic Encephalopathy Trial