<i>Nicotiana benthamiana</i> &#945;-galactosidase A1.1 can functionally complement human &#945;-galactosidase A deficiency associated with Fabry disease.

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

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

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.

Afiliação

Kytidou K; From the Departments of Medical Biochemistry.
Beekwilder J; the Plant Sciences Group, Wageningen University and Research, Droevendaalsesteeg 1, 6708 PB Wageningen, and.
Artola M; Bio-organic Synthesis, and.
van Meel E; From the Departments of Medical Biochemistry.
Wilbers RHP; the Plant Sciences Group, Wageningen University and Research, Droevendaalsesteeg 1, 6708 PB Wageningen, and.
Moolenaar GF; Cloning and Protein Purification Facility, Leiden Institute of Chemistry, Einsteinweg 55, 2333 CC Leiden.
Goosen N; Cloning and Protein Purification Facility, Leiden Institute of Chemistry, Einsteinweg 55, 2333 CC Leiden.
Ferraz MJ; From the Departments of Medical Biochemistry.
Katzy R; From the Departments of Medical Biochemistry.
Voskamp P; Biophysical Structural Chemistry and.
Florea BI; Bio-organic Synthesis, and.
Hokke CH; the Department of Parasitology, Centre of Infectious Diseases, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands.
Overkleeft HS; Bio-organic Synthesis, and.
Schots A; the Plant Sciences Group, Wageningen University and Research, Droevendaalsesteeg 1, 6708 PB Wageningen, and.
Bosch D; the Plant Sciences Group, Wageningen University and Research, Droevendaalsesteeg 1, 6708 PB Wageningen, and.
Pannu N; Biophysical Structural Chemistry and.
Aerts JMFG; From the Departments of Medical Biochemistry, j.m.f.g.aerts@lic.leidenuniv.nl.

J Biol Chem ; 293(26): 10042-10058, 2018 06 29.

Article em En | MEDLINE | ID: mdl-29674318

Assuntos

Doença de Fabry/enzimologia; Nicotiana/enzimologia; alfa-Galactosidase/metabolismo; Biocatálise; Membrana Celular/metabolismo; Doença de Fabry/patologia; Feminino; Fibroblastos/metabolismo; Humanos; Masculino; Nicotiana/citologia; alfa-Galactosidase/genética

Palavras-chave

Fabry disease; Nicotiana benthamiana; enzyme inhibitor; enzyme purification; enzyme structure; enzyme-replacement therapy; fluorescence; glycolipid; glycoside hydrolase; glycosphingolipid; glycosphingolipids; homologue; human; lysosomal storage disorder; plant; protein expression; recombinant enzyme; α-galactosidase

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Nicotiana / Doença de Fabry / Alfa-Galactosidase Tipo de estudo: Risk_factors_studies Limite: Female / Humans / Male Idioma: En Ano de publicação: 2018 Tipo de documento: Article

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