Production of a functional human acid maltase in tobacco seeds: Biochemical analysis, uptake by human GSDII cells, and in vivo studies in GAA knockout mice

Applied Biochemistry and Biotechnology

Volumn 171, Issue 4, 2013, Pages 916-926

  Martiniuk, Frank ^a,b   Reggi, Serena ^c   Tchou Wong, Kam Meng ^a   Rom, William N ^a   Busconi, Matteo ^d   Fogher, Corrado ^c  

^a NEW YORK UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b IPRO Inc   (United States)

^c Plantechno Srl   (Italy)

^d UNIVERSITÀ CATTOLICA DEL SACRO CUORE   (Italy)

Author keywords

Enzyme replacement; Pompe's disease; Recombinant human acid maltase; Transgenic tobacco plants

Indexed keywords

ALPHA GLUCOSIDASE; BIOCHEMICAL ANALYSIS; ENZYME REPLACEMENT THERAPY; GENETIC DEFICIENCIES; MANNOSE 6 PHOSPHATES; RECOMBINANT HUMAN ACID MALTASE; TRANSGENIC TOBACCO PLANTS; WHITE BLOOD CELLS;

BIOACTIVITY; CELL CULTURE; DEFECTS; DIGITAL STORAGE; ENZYMES; ION EXCHANGE; MACHINERY; MAMMALS; PHOSPHORYLATION; SEED;

GALLIUM ALLOYS;

BETA CONGLYCININ; COMPLEMENTARY DNA; GLUCAN 1,4 ALPHA GLUCOSIDASE; GLYCININ; RECOMBINANT PROTEIN; UNCLASSIFIED DRUG;

AMINO ACID SEQUENCE; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME SYNTHESIS; FIBROBLAST; GENE SEQUENCE; GLYCOSYLATION; HUMAN; HUMAN CELL; LEUKOCYTE; MALE; MOUSE; NONHUMAN; NUCLEOTIDE SEQUENCE; PLANT SEED; PROMOTER REGION; PROTEIN EXPRESSION; PROTEIN PHOSPHORYLATION; PROTEIN PROCESSING; TOBACCO;

GLYCINE MAX; MAMMALIA; MUS; NICOTIANA OBTUSIFOLIA; NICOTIANA TABACUM;

ALPHA-GLUCOSIDASES; ANIMALS; FEMALE; GLUCAN 1,4-ALPHA-GLUCOSIDASE; GLYCOGEN STORAGE DISEASE TYPE II; HUMANS; MICE; MICE, INBRED BALB C; MICE, KNOCKOUT; PLANTS, GENETICALLY MODIFIED; SEEDS; TOBACCO;

EID: 84885179052     PISSN: 02732289     EISSN: 15590291     Source Type: Journal    
DOI: 10.1007/s12010-013-0367-z     Document Type: Article

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