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Odiparcil is a β-D-thioxyloside derivative and driver of circulating glycosaminoglycan (GAG) synthesis.1,2 It increases the secretion of total sulfated GAGs in bovine aortic endothelial (BAE) cells when used at a concentration of 10 µM.1 Odiparcil (10 µM) reduces intracellular chondroitin sulfate levels in primary skin fibroblasts isolated from patients with mucopolysaccharidosis type VI (MPS VI), an inborn error of metabolism characterized by a deficiency in the degradation of specific GAGs. Dietary administration of odiparcil (4.5 g/kg of chow) increases urinary excretion of sulfated GAGs and decreases hepatic and renal accumulation of sulfated GAGs in a mouse model of MPS VI induced by a homozygous gene mutation in the Arsb gene, which encodes the enzyme arylsulfatase B. Odiparcil reduces thrombus weight in a rat model of thrombosis (ED50 = 3.2 mg/kg).2
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1. Odiparcil, a potential glycosaminoglycans clearance therapy in mucopolysaccharidosis VI-
2. A comparison of the β-