المؤلفون: Deng, Mulan, Zhou, Hongyu, He, Shaomei, Qiu, Haoheng, Wang, Yanping, Zhao, April Yuanyi, Mu, Yunping^{Aff1, IDs13023023028940_cor7}, Li, Fanghong^{Aff1, IDs13023023028940_cor8}, Zhao, Allan Zijian^{Aff1, IDs13023023028940_cor9}

المصدر: Orphanet Journal of Rare Diseases. 18(1)

المؤلفون: Mulan Deng, Hongyu Zhou, Shaomei He, Haoheng Qiu, Yanping Wang, April Yuanyi Zhao, Yunping Mu, Fanghong Li, Allan Zijian Zhao

المصدر: Orphanet Journal of Rare Diseases, Vol 18, Iss 1, Pp 1-13 (2023)

مصطلحات موضوعية: Fabry disease (FD), Adeno-associated viral 2/8 (AAV2/8), α-galactosidase A (α-Gal A), Globotriaosylceramide (Gb3), Globotriaosylsphingosine (Lyso-Gb3), Lysosomal storage diseases (LSDs), Medicine

وصف الملف: electronic resource

Relation: https://doaj.org/toc/1750-1172

URL الوصول: https://doaj.org/article/db59864a565d4f54ad60dc9bf0bd89de

المؤلفون: Solvey Pollmann, David Scharnetzki, Dominique Manikowski, Malte Lenders, Eva Brand

المصدر: Frontiers in Immunology, Vol 12 (2021)

مصطلحات موضوعية: Fabry disease (FD), endothelial dysfunction, heparanase, angiopoietin-1-receptor, globotriaosylsphingosine (lyso-Gb3), NF-κB, Immunologic diseases. Allergy, RC581-607

وصف الملف: electronic resource

Relation: https://www.frontiersin.org/articles/10.3389/fimmu.2021.789142/full; https://doaj.org/toc/1664-3224

URL الوصول: https://doaj.org/article/496b45111a274907bee2fcb13ab63526

المؤلفون: Aizeddin A. Mhanni, Christiane Auray-Blais, Michel Boutin, Alie Johnston, Kaye LeMoine, Jill Patterson, Johannes M.F.G. Aerts, Michael L. West, Cheryl Rockman-Greenberg

المصدر: Molecular Genetics and Metabolism Reports, Vol 24, Iss , Pp 100618- (2020)

مصطلحات موضوعية: Fabry disease, Agalsidase antibodies, Enzyme replacement therapy, Globotriaosylceramide (Gb3), Globotriaosylsphingosine (Lyso-Gb3), Medicine (General), R5-920, Biology (General), QH301-705.5

وصف الملف: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2214426920300641; https://doaj.org/toc/2214-4269

URL الوصول: https://doaj.org/article/23d63e924b9b4c13a433ac1a3d2aa15a

المؤلفون: Kazuya Tsuboi, Hiroshi Yamamoto

المصدر: BMC Pharmacology and Toxicology, Vol 18, Iss 1, Pp 1-9 (2017)

مصطلحات موضوعية: Fabry disease, Enzyme replacement therapy (ERT), Agalsidase Alfa, Globotriaosylceramide (Gb3), Globotriaosylsphingosine (lyso-Gb3), Therapeutics. Pharmacology, RM1-950, Toxicology. Poisons, RA1190-1270

وصف الملف: electronic resource

Relation: http://link.springer.com/article/10.1186/s40360-017-0152-7; https://doaj.org/toc/2050-6511

URL الوصول: https://doaj.org/article/f392deeb76514059ab2a5e0d28dce294

المؤلفون: Eva Brand, Malte Lenders, Dominique Manikowski, Solvey Pollmann, David Scharnetzki

المصدر: Frontiers in Immunology
Frontiers in Immunology, Vol 12 (2021)

مصطلحات موضوعية: Adult, Male, medicine.medical_specialty, angiopoietin-1-receptor, 1-Deoxynojirimycin, THP-1 Cells, Immunology, Anti-Inflammatory Agents, Globotriaosylceramide, Inflammation, Glycocalyx, endothelial dysfunction, NF-κB, heparanase, chemistry.chemical_compound, Vascular Stiffness, Internal medicine, medicine, Lysosomal storage disease, Humans, Immunology and Allergy, Enzyme Replacement Therapy, Genetic Predisposition to Disease, Heparanase, Prospective Studies, Endothelial dysfunction, Original Research, Aged, globotriaosylsphingosine (lyso-Gb3), Endothelial Cells, Enzyme replacement therapy, RC581-607, Middle Aged, medicine.disease, Fabry disease, Coculture Techniques, Phenotype, Endocrinology, chemistry, Case-Control Studies, alpha-Galactosidase, Mutation, Fabry disease (FD), Fabry Disease, Endothelium, Vascular, Immunologic diseases. Allergy, medicine.symptom

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8f41bb2e1b7957313517dd1c24774cd1
https://doi.org/10.3389/fimmu.2021.789142

المؤلفون: Takahiro Tsukimura, Satoshi Gojo, Masashi Yamanami, Hideki Maeda, Daisuke Kami, Tadayasu Togawa, Hitoshi Sakuraba

المصدر: Cell Transplantation, Vol 29 (2020)
Cell Transplantation

مصطلحات موضوعية: Cell, Biomedical Engineering, Globotriaosylceramide, lcsh:Medicine, CellSaic, chemistry.chemical_compound, Mice, In vivo, medicine, Animals, Humans, Enzyme Replacement Therapy, cell transplantation, globotriaosylceramide (Gb3), Transplantation, Fabry disease, globotriaosylsphingosine (lyso-Gb3), business.industry, Trihexosylceramides, lcsh:R, Glomerulosclerosis, Cell Biology, Enzyme replacement therapy, medicine.disease, Embryonic stem cell, Immunohistochemistry, Rats, medicine.anatomical_structure, chemistry, alpha-galactosidase (GLA in humans and Gla in mice), Rats, Inbred Lew, spheroid, Heart failure, alpha-Galactosidase, Cancer research, Original Article, Collagen, business, Peptides

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::107376d8efc2575a2e4e1a51ed7e53e5
https://doaj.org/article/d98bb0111921488aabf9a57817fe7148

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المؤلفون: Jill Patterson, Kaye LeMoine, Alie J. Johnston, Christiane Auray-Blais, Michael West, Cheryl Rockman-Greenberg, Johannes M. F. G. Aerts, Michel Boutin, Aizeddin A. Mhanni

المصدر: Molecular Genetics and Metabolism Reports
Molecular Genetics and Metabolism Reports, Vol 24, Iss, Pp 100618-(2020)
Molecular Genetics and Metabolism Reports, 24, 100618. ELSEVIER

مصطلحات موضوعية: medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, Globotriaosylceramide, Case Report, Disease, Malaise, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Endocrinology, Internal medicine, Agalsidase antibodies, Genetics, Medicine, Globotriaosylceramide (Gb3), Molecular Biology, lcsh:QH301-705.5, 0303 health sciences, Creatinine, lcsh:R5-920, Fabry disease, biology, business.industry, 030305 genetics & heredity, nutritional and metabolic diseases, Enzyme replacement therapy, medicine.disease, chemistry, lcsh:Biology (General), biology.protein, Premedication, Antibody, medicine.symptom, business, lcsh:Medicine (General), 030217 neurology & neurosurgery, Globotriaosylsphingosine (Lyso-Gb3)

وصف الملف: application/pdf

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1660426923842e69b38f8fdf959959b3
http://europepmc.org/articles/PMC7322173

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