Nicotiana benthamiana α-galactosidase A1.1 can functionally complement human α-galactosidase A deficiency associated with Fabry disease

α-Galactosidases (EC 3.2.1.22) are retaining glycosidases that cleave terminal α-linked galactose residues from glycoconjugate substrates. α-Galactosidases take part in the turnover of cell wall–associated galactomannans in plants and in the lysosomal degradation of glycosphingolipids in animals. De...

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Udgivet i:J Biol Chem
Main Authors: Kytidou, Kassiani, Beekwilder, Jules, Artola, Marta, van Meel, Eline, Wilbers, Ruud H. P., Moolenaar, Geri F., Goosen, Nora, Ferraz, Maria J., Katzy, Rebecca, Voskamp, Patrick, Florea, Bogdan I., Hokke, Cornelis H., Overkleeft, Herman S., Schots, Arjen, Bosch, Dirk, Pannu, Navraj, Aerts, Johannes M. F. G.
Format: Artigo
Sprog:Inglês
Udgivet: American Society for Biochemistry and Molecular Biology 2018
Fag:
Enzymology
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6028973/
https://ncbi.nlm.nih.gov/pubmed/29674318
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.RA118.001774
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