产品描述:
Adenylosuccinic acid is a purine nucleotide and an intermediate in the purine nucleotide cycle.{52338} It is converted into adenosine 5'-monophosphate (AMP; Item No. 21094) and fumarate by adenylosuccinate lyase (ADSL) in the cytosol. Adenylosuccinic acid (10 µM) inhibits calcium-induced activation of non-selective cation channels in isolated rat brown adipocytes in a patch-clamp assay.{52339} It induces contractions in isolated guinea pig uterus strips, which express adenosine receptors, when used at a concentration of 100 µM.{52341} Adenylosuccinic acid (10 µM) increases glucose-induced insulin exocytosis in INS-1 832/13 insulinoma cells.{52340} In vivo, adenylosuccinic acid (3,000 µg/ml in the drinking water) decreases muscle damage and pseudohypertrophy in a dystrophin-deficient mdx mouse model of Duchenne muscular dystrophy (DMD).{51595} Urine levels of adenylosuccinic acid are increased in patients with ADSL deficiency, an inborn error of metabolism characterized by various neurological symptoms, including intellectual disability and seizures, as well as respiratory failure.{51594}