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Description:Physiologically, argininosuccinate lyase catalyzes the conversion of argininosuccinic acid into arginine and fumarate. The enzyme is one of the urea cycle enzymes. The deficiency of argininosuccinate lyase causes argininosuccinic aciduria. This assay measures the ability of the enzyme to catalyze the reverse reaction, i.e. the formation of argininosuccinate from arginine and 14C-fumaric acid. Products and substrates are separated on an ion exchange column. Specimen Requirements:This assay can be performed on red blood cells, cultured skin fibroblasts and virtually any tissue. For analysis of red blood cells, draw 3-5 ml in a yellow top (ACD) tube. Sample must arrive at our location within 48 hrs. If liver is to be used, it must be rapidly frozen following collection. Do not add any liquid to the specimen. Send >10 mg of tissue on dry ice by overnight carrier. For fibroblasts, send two T25 flasks of confluent cells in an insulated container by overnight carrier. Contact the laboratory for information regarding prenatal diagnosis studies. Normal Range:Liver: 350-1200 µmoles/hr/gm
tissue Turnaround Time: Amniotic Cell Culture, Cultured Chorionic Villus Cells: 3
weeks CPT Codes and Prices:Amniotic Cell Culture: 88235x4,
82658x2, 82491x2, 88240x2, 84155x2, 83912x2, 83901x8, 83894x8, 83891x2, 82492,
82139 Shipping InformationForms:>> Biochemical
Requisition Test Codes:Amniotic Cell Culture: 4521 |