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Characterization and downstream mannose phosphorylation of human recombinant α-L-iduronidase produced in Arabidopsis complex glycan-deficient (cgl) seeds

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-acetylglucosa...

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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BlackWell Publishing Ltd 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4030584/
https://www.ncbi.nlm.nih.gov/pubmed/23898885
http://dx.doi.org/10.1111/pbi.12096