Human a-mannosidase produced in transgenic tobacco plants is processed in human alpha-mannosidosis cell lines (Articolo in rivista)

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Label
  • Human a-mannosidase produced in transgenic tobacco plants is processed in human alpha-mannosidosis cell lines (Articolo in rivista) (literal)
Anno
  • 2011-01-01T00:00:00+01:00 (literal)
Alternative label
  • De Marchis Francesca; Balducci Chiara; Pompa Andrea; Hilde M. F. Riise Stensland; Guaragno Marco; Pagiotti Rita; Menghini AR; Persichetti Emanuele; Beccari Tommaso; Bellucci Michele; (2011)
    Human a-mannosidase produced in transgenic tobacco plants is processed in human alpha-mannosidosis cell lines
    in Plant biotechnology journal (Print)
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • De Marchis Francesca; Balducci Chiara; Pompa Andrea; Hilde M. F. Riise Stensland; Guaragno Marco; Pagiotti Rita; Menghini AR; Persichetti Emanuele; Beccari Tommaso; Bellucci Michele; (literal)
Pagina inizio
  • 1061 (literal)
Pagina fine
  • 1073 (literal)
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  • 9 (literal)
Rivista
Note
  • PubMe (literal)
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  • De Marchis Francesca; Institute of Plant Genetics, Italian National Council of Research (CNR), Perugia, Italy Balducci Chiara; Department of Economics and Food Science, University of Perugia, Italy Pompa Andrea; Institute of Plant Genetics, Italian National Council of Research (CNR), Perugia, Italy Hilde M. F. Riise Stensland; Department of Medical Genetics, Division of Child and Adolescent Health, University Hospital of North-Norway, Tromsø, Norway Guaragno Marco; Institute of Plant Genetics, Italian National Council of Research (CNR), Perugia, Italy Pagiotti Rita; Department of Applied Biology, University of Perugia, Italy Menghini AR; Department of Experimental Medicine and Biochemical Sciences, University of Perugia, Italy Persichetti Emanuele; Department of Experimental Medicine and Biochemical Sciences, University of Perugia, Italy Beccari Tommaso; Department of Experimental Medicine and Biochemical Sciences, University of Perugia, Italy Bellucci Michele; Institute of Plant Genetics, Italian National Council of Research (CNR), Perugia, Italy (literal)
Titolo
  • Human a-mannosidase produced in transgenic tobacco plants is processed in human alpha-mannosidosis cell lines (literal)
Abstract
  • Deficiency in human lysosomal a-mannosidase (MAN2B1) results in a-mannosidosis, a lysosomal storage disorder; patients present a wide range of neurological, immunological, and skeletal symptoms caused by a multisystemic accumulation of mannose-containing oligosaccharides. Here, we describe the expression of recombinant MAN2B1 both transiently in Nicotiana benthamiana leaves and in the leaves and seeds of stably transformed N. tabacum plants. After purification from tobacco leaves, the recombinant enzyme was found to be N-glycosylated and localized in vacuolar compartments. In the fresh leaves of tobacco transformants, MAN2B1 was measured at 10 200 units / kg, and the purified enzyme from these leaves had a specific activity of 32-45 U / mg. Furthermore, tobacco-produced MAN2B1 was biochemically similar to the enzyme purified from human tissues, and it was internalized and processed by a-mannosidosis fibroblast cells. These results strongly indicate that plants can be considered a promising expression system for the production of recombinant MAN2B1 for use in enzyme replacement therapy. (literal)
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