Glycosylation-independent lysosomal targeting of acidα-glucosidase enhances muscle glycogen clearance in pompe mice

Journal of Biological Chemistry

Volumn 288, Issue 3, 2013, Pages 1428-1438

  Maga, John A ^a   Zhou, Jianghong ^a,d   Kambampati, Ravi ^a   Peng, Susan ^a   Wang, Xu ^b   Bohnsack, Richard N ^c   Thomm, Angela ^a,e   Golata, Sarah ^a,f   Tom, Peggy ^a,g   Dahms, Nancy M ^c   Byrne, Barry J ^b   LeBowitz, Jonathan H ^a,h  

^a ZyStor Therapeutics Inc   (United States)

^b UNIVERSITY OF FLORIDA COLLEGE OF MEDICINE   (United States)

^c MEDICAL COLLEGE OF WISCONSIN   (United States)

^d Oncode Med Inc   (United States)

^e PhysioGenix Inc   (United States)

^f Benz Oil Inc   (United States)

^g Cytometix   (United States)

^h BIOMARIN PHARMACEUTICAL INC   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CATION-INDEPENDENT MANNOSE 6-PHOSPHATE RECEPTORS; CHIMERIC ENZYMES; CLINICAL STUDY; ENZYME REPLACEMENT THERAPY; GLUCOSIDASE; HIGH AFFINITY; INSULIN-LIKE GROWTH FACTOR; MOUSE MODELS; MYOBLASTS; POMPE DISEASE; SKELETAL MUSCLE; TARGETING SYSTEMS;

DISEASE CONTROL; ENZYMES; ESTERIFICATION; GLYCOSYLATION; MAMMALS; MUSCLE; PATIENT TREATMENT; POSITIVE IONS;

GALLIUM ALLOYS;

BMN 701; GLUCAN 1,4 ALPHA GLUCOSIDASE; GLYCOGEN; RECOMBINANT GLUCAN 1,4 ALPHA GLUCOSIDASE; SOMATOMEDIN B; SOMATOMEDIN B RECEPTOR; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CHO CELL; CONTROLLED STUDY; ENZYME ACTIVITY; GLYCOGEN MUSCLE LEVEL; GLYCOGEN STORAGE DISEASE TYPE 2; GLYCOSYLATION INDEPENDENT LYSOSOMAL TARGETING; IN VITRO STUDY; INTERNALIZATION; LYSOSOME; MOUSE; MYOBLAST; NONHUMAN; PRIORITY JOURNAL; PROTEIN TARGETING; RAT;

ANIMALS; BIOLOGICAL TRANSPORT; DISEASE MODELS, ANIMAL; DRUG DELIVERY SYSTEMS; ENZYME REPLACEMENT THERAPY; GLUCAN 1,4-ALPHA-GLUCOSIDASE; GLYCOGEN; GLYCOGEN STORAGE DISEASE TYPE II; GLYCOSYLATION; HALF-LIFE; HEK293 CELLS; HUMANS; INSULIN-LIKE GROWTH FACTOR II; KINETICS; LYSOSOMES; MICE; MUSCLE, SKELETAL; MUTANT CHIMERIC PROTEINS; MYOBLASTS; PLASMIDS; RECEPTOR, IGF TYPE 2; TRANSFECTION;

MUS;

EID: 84872707665     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M112.438663     Document Type: Article

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