Article
Characterization and downstream mannose phosphorylation of human recombinant α-L-iduronidase produced in Arabidopsis complex glycan-deficient (cgl) seeds.
Plant biotechnology journal - 1 Dec 2013
He Xu, Pierce Owen, Haselhorst Thomas, von Itzstein Mark, Kolarich Daniel, Packer Nicolle H, Gloster Tracey M, Vocadlo David J, Qian Yi, Brooks Doug, Kermode Allison R
Abstract excerpt
Mucopolysaccharidosis (MPS) I is a lysosomal storage disease caused by a deficiency of α-L-iduronidase (IDUA) (EC 3.2.1.76); enzyme replacement therapy is the conventional treatment for this genetic disease. Arabidopsis cgl mutants are characterized by a deficiency of the activity of N-acetylglucosaminyl transferase I (EC 2.4.1.101), the first enzyme in the pathway of hybrid and complex N-glycan biosynthesis. To...
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