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Bioconjugate Chemistry, ISSN 1043-1802, 03/2018, Volume 29, Issue 3, pp. 649 - 656
Enzymes are attractive as immunotherapeutics because they can catalyze shifts in the local availability of immunostimulatory and immunosuppressive signals.... 
BIOCHEMISTRY & MOLECULAR BIOLOGY | BIOCHEMICAL RESEARCH METHODS | CHEMISTRY, ORGANIC | LYSOSOMAL STORAGE DISEASES | BLOOD-BRAIN-BARRIER | CHEMISTRY, MULTIDISCIPLINARY | ANTITUMOR IMMUNE-RESPONSES | ACUTE LYMPHOBLASTIC-LEUKEMIA | REPLACEMENT THERAPY | FUSION PROTEIN | ADENOSINE-DEAMINASE DEFICIENCY | POLYETHYLENE-GLYCOL | EXTRACELLULAR-SUPEROXIDE DISMUTASE | TARGETED DELIVERY | Glucosylceramidase - chemistry | alpha-Galactosidase - therapeutic use | Immunotherapy - methods | Humans | Anti-Inflammatory Agents - immunology | Antineoplastic Agents - therapeutic use | Asparaginase - chemistry | Gaucher Disease - therapy | Enzymes, Immobilized - immunology | Enzyme Therapy - methods | Immunoconjugates - immunology | alpha-Galactosidase - chemistry | Neoplasms - therapy | Lysosomal Storage Diseases - immunology | Anti-Inflammatory Agents - therapeutic use | Immunoconjugates - therapeutic use | Fabry Disease - immunology | alpha-Galactosidase - immunology | Asparaginase - therapeutic use | Enzymes, Immobilized - therapeutic use | Enzymes, Immobilized - chemistry | Biocatalysis | Lysosomal Storage Diseases - therapy | Antineoplastic Agents - immunology | Glucosylceramidase - immunology | Gaucher Disease - immunology | Glycosylation | Inflammation - immunology | Antineoplastic Agents - chemistry | Immunoconjugates - chemistry | Asparaginase - immunology | Inflammation - therapy | Animals | Anti-Inflammatory Agents - chemistry | Glucosylceramidase - therapeutic use | Neoplasms - immunology | Fabry Disease - therapy | Immunohistochemistry | Enzymes | Immunostimulation | Immobilization | Recovery of function | Radioactive half-life | Biocatalysts | Immunity | Substrates | Organic chemistry | Chemical modification | Immunosuppression | Immunotherapy | Catalysis | Half life
Journal Article
Biochemical Journal, ISSN 0264-6021, 09/2007, Volume 406, Issue 2, pp. 285 - 295
Fabry disease is a lysosomal storage disorder caused by the deficiency of alpha-Gal A (alpha-galactosidase A) activity. In order to understand the molecular... 
Fabry disease | Endoplasmic reticulum-associated degradation (ERAD) | α-galactosidase A | Active-site-specific chaperone (ASSC) | Protein misfolding | 1-deoxygalactonojirimycin (DGJ) | BIOCHEMISTRY & MOLECULAR BIOLOGY | ATYPICAL VARIANT | LYSOSOMAL STORAGE | QUALITY-CONTROL | NATURAL-HISTORY | active-site-specific chaperone (ASSC) | 1-deoxy-galactonojirimycin (DGJ) | endoplasmic reticulum-associated degradation (ERAD) | CHAPERONE THERAPY | POINT MUTATIONS | GENE | protein misfolding | alpha-galactosidase A | REPLACEMENT THERAPY | ENDOPLASMIC-RETICULUM | PROTEINS | Protein Structure, Tertiary | alpha-Galactosidase - genetics | Fabry Disease - classification | Fabry Disease - genetics | Humans | Cells, Cultured | Enzyme Stability | Cercopithecus aethiops | Models, Molecular | alpha-Galactosidase - metabolism | Fabry Disease - enzymology | Mutation - genetics | Fabry Disease - pathology | Protein Transport | Gene Expression Regulation, Enzymologic | alpha-Galactosidase - chemistry | 1-Deoxynojirimycin - pharmacology | alpha-Galactosidase - isolation & purification | Animals | Cell Communication - drug effects | 1-Deoxynojirimycin - analogs & derivatives | Kinetics | Binding Sites | Hydrogen-Ion Concentration | DGJ, 1-deoxygalactonojirimycin | 4MU-α-Gal, 4MU α-D-galactopyranoside | GLA, α-Gal A gene | ERAD, endoplasmic reticulum-associated degradation | 4MU, 4-methylumbelliferyl | α-Gal A, α-galactosidase A | DMEM, Dulbecco's modified Eagle's medium | ASSC, active-site-specific chaperone | ER, endoplasmic reticulum | FCS, fetal calf serum
Journal Article
Journal Article
Journal Article
Autophagy, ISSN 1554-8627, 11/2010, Volume 6, Issue 8, pp. 1078 - 1089
Journal Article
Stroke, ISSN 0039-2499, 08/2018, Volume 49, Issue 8, pp. 1977 - 1980
BACKGROUND AND PURPOSE—Absent or diminished α-galactosidase A (GLA) and acid α-glucosidase (GAA) enzyme activity are core features of Fabry and Pompe disease,... 
inflammation | sphingolipids | dilatation | PERIPHERAL VASCULAR DISEASE | lysosomes | FABRY DISEASE | hypertension | CLINICAL NEUROLOGY | PARKINSONS-DISEASE | lysosomal dysfunction | brain arterial diameters | Dolichoectasia | lysosomal enzymes
Journal Article
Journal Article
2013, AHRQ publication, Volume no. 12(13)-EHC154-EF
BACKGROUND: Lysosomal storage diseases (LSDs) comprise about 50 unique monogenic autosomal or X-linked diseases with an estimated combined incidence of 1 in... 
Enzyme Replacement Therapy | drug therapy | Lysosomal Storage Diseases | methods
Web Resource
Nature Reviews Molecular Cell Biology, ISSN 1471-0072, 09/2018, Volume 19, Issue 9, pp. 563 - 578
Journal Article