ABSTRACT

Description: Laronidase is a polymorphic variant of human α-Liduronidase (EC 3.2.1.76), a lysosomal hydrolase that catalyses the hydrolysis of terminal α-L-iduronic acid residues of dermatan sulphate and heparan sulphate. The mature enzyme is a 628-amino acid, 83-kDa monomeric glycoprotein, containing 6 N-linked oligosaccharide side chains. It is provided as a concentrate solution for intravenous infusion. The laronidase concentration is 0.58 mg/ml, with a specific activity of approximately 172 U/mg.