المؤلفون: Joaquin Seras‐Franzoso, Zamira V. Díaz‐Riascos, José Luis Corchero, Patricia González, Natalia García‐Aranda, Mònica Mandaña, Roger Riera, Ana Boullosa, Sandra Mancilla, Alba Grayston, Marc Moltó‐Abad, Elena Garcia‐Fruitós, Rosa Mendoza, Guillem Pintos‐Morell, Lorenzo Albertazzi, Anna Rosell, Josefina Casas, Antonio Villaverde, Simó Schwartz Jr, Ibane Abasolo

المصدر: Journal of Extracellular Vesicles, Vol 10, Iss 5, Pp n/a-n/a (2021)

مصطلحات موضوعية: alpha‐galactosidase A, drug delivery, enzyme replacement therapy, Fabry disease, lysosomal storage disorders, N‐sulfoglucosamine sulfohydrolase, Cytology, QH573-671

وصف الملف: electronic resource

Relation: https://doaj.org/toc/2001-3078

URL الوصول: https://doaj.org/article/017c9b6b0e7544dbad5fbbce1391a4b6

المؤلفون: Lauren S. Whyte, John J. Hopwood, Kim M. Hemsley, Adeline A. Lau

المصدر: Molecular Genetics and Metabolism Reports, Vol 5, Iss C, Pp 60-62 (2015)

مصطلحات موضوعية: N-sulfoglucosamine sulfohydrolase, Enzyme activity, Fluorometry, Sanfilippo, Mucopolysaccharidosis, Medicine (General), R5-920, Biology (General), QH301-705.5

وصف الملف: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2214426915300409; https://doaj.org/toc/2214-4269

URL الوصول: https://doaj.org/article/e35af6316e95450f90480b5c0a4939dd

المؤلفون: Rosa Mendoza, Zamira V. Díaz-Riascos, Sandra Mancilla, Lorenzo Albertazzi, Natalia García-Aranda, Ana Boullosa, Antonio Villaverde, Monica Mandaña, Patricia González, Ibane Abasolo, Anna Rosell, Guillem Pintos-Morell, José Luis Corchero, Josefina Casas, Simó Schwartz, Alba Grayston, Joaquin Seras-Franzoso, Roger Riera, Elena García-Fruitós, Marc Moltó-Abad

المساهمون: Institut Català de la Salut, [Seras-Franzoso J, González P, Schwartz S Jr] Drug Delivery & Targeting, CIBBIM-Nanomedicine, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. Networking Research Center on Bioengineering, Biomaterials and Nanomedicine (CIBER-BBN), Barcelona, Spain. [Díaz-Riascos ZV, García-Aranda N, Mandaña M, Boullosa A, Mancilla S, Abasolo I] Drug Delivery & Targeting, CIBBIM-Nanomedicine, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. Networking Research Center on Bioengineering, Biomaterials and Nanomedicine (CIBER-BBN), Barcelona, Spain. Functional Validation & Preclinical Research (FVPR), CIBBIM-Nanomedicine, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. [Corchero JL] Networking Research Center on Bioengineering, Biomaterials and Nanomedicine (CIBER-BBN), Barcelona, Spain. Institut de Biotecnologia i de Biomedicina (IBB) and Department of Genetics and Microbiology, Universitat Autònoma de Barcelona, Bellaterra, Spain. [Grayston A, Rosell A] Neurovascular Research Laboratory, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. [Moltó-Abad M, Pintos-Morell G] Drug Delivery & Targeting, CIBBIM-Nanomedicine, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. Division of Rare Diseases, Reference Center for Hereditary Metabolic Disorders (CSUR, XUEC, MetabERN, and CIBER-ER). Vall d’Hebron Hospital Universitari, Barcelona, Spain, Vall d'Hebron Barcelona Hospital Campus

المصدر: Dipòsit Digital de Documents de la UAB
Universitat Autònoma de Barcelona
Journal of Extracellular Vesicles, Vol 10, Iss 5, Pp n/a-n/a (2021)
Scientia
Journal of Extracellular Vesicles
Digital.CSIC. Repositorio Institucional del CSIC
instname

مصطلحات موضوعية: Male, 0301 basic medicine, Hydrolases, Cell, Sanfilippo syndrome, Otros calificadores::Otros calificadores::Otros calificadores::/enzimología [Otros calificadores], law.invention, Mice, chemistry.chemical_compound, 0302 clinical medicine, law, Cells::Cellular Structures::Extracellular Space::Extracellular Vesicles [ANATOMY], Cloning, Molecular, Research Articles, Mice, Knockout, chemistry.chemical_classification, Trihexosylceramides, Brain, Enzyme replacement therapy, Recombinant Proteins, Cell biology, N-sulfoglucosamine sulfohydrolase, medicine.anatomical_structure, Metabolisme - Trastorns, 030220 oncology & carcinogenesis, Recombinant DNA, Pharmaceutical Vehicles, Research Article, Histology, Globotriaosylceramide, CHO Cells, Nutritional and Metabolic Diseases::Metabolic Diseases::Metabolism, Inborn Errors::Lysosomal Storage Diseases [DISEASES], Lysosomal storage disorders, Enzims extracel·lulars - Ús terapèutic, lysosomal storage disorders, Extracellular Vesicles, 03 medical and health sciences, Cricetulus, In vivo, medicine, Animals, Humans, Alpha-galactosidase A, enfermedades nutricionales y metabólicas::enfermedades metabólicas::alteraciones congénitas del metabolismo::enfermedades por almacenamiento lisosómico [ENFERMEDADES], Fabry disease, QH573-671, alpha‐galactosidase A, células::estructuras celulares::espacio extracelular::vesículas extracelulares [ANATOMÍA], N‐sulfoglucosamine sulfohydrolase, Cell Biology, medicine.disease, Lysosomal Storage Diseases, Mice, Inbred C57BL, HEK293 Cells, 030104 developmental biology, Enzyme, chemistry, alpha-Galactosidase, Drug delivery, Lysosomes, Cytology, Other subheadings::Other subheadings::Other subheadings::/enzymology [Other subheadings]

وصف الملف: application/pdf

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::702cb6a58bfe0e56afbde20b3d5154b2
http://hdl.handle.net/10261/234882

المؤلفون: Nicole Muschol, Marcel du Moulin, Luise Ammer, Anja Köhn, Cornelia Rudolph, Sarah Kabisch, Lorenz Grigull

المصدر: Molecular Genetics and Metabolism Reports
Molecular Genetics and Metabolism Reports, Vol 23, Iss, Pp-(2020)

مصطلحات موضوعية: Pediatrics, medicine.medical_specialty, DQ, developmental quotient, GAG, Glykosaminoglycans, medicine.medical_treatment, Sanfilippo syndrome, Natural history, ATG, antithymocyte globulin, Mucopolysaccharidosis type III, Hematopoietic stem cell transplantation, UCBT, umbilical cord blood-derived hematopoietic stem cell transplantation, VABS-II, Vineland Adaptive Behavior Scales, HSCT, hematopoietic stem cell transplantation, Endocrinology, Quality of life, Genetics, medicine, Dementia, Global developmental delay, ICLD, International Center for Lysosomal Disorders, Molecular Biology, Mucopolysaccharidosis Type IIIA, lcsh:QH301-705.5, lcsh:R5-920, business.industry, MPS IIIA, mucopolysaccharidosis type IIIA, MPS IIIB, mucopolysaccharidosis type IIIB, SGSH, N-sulfoglucosamine sulfohydrolase, Stem cell transplantation, TDS, total disability score, medicine.disease, MPS IH, mucopolysaccharidosis type I (Hurler syndrome), FPSS, four point scoring system, AEq, age-equivalent score, lcsh:Biology (General), MPS IIIA, HSCT, y, years, business, lcsh:Medicine (General), Avg., Average, Research Paper

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a778c89879896cc236478fb88a53708d
http://europepmc.org/articles/PMC7093801

المؤلفون: Joost H.A. Folgering, Maria Eriksson, Lars Bergquist, Gudrun Andersson, Juliette Janson

المصدر: Molecular Genetics and Metabolism Reports, Vol 22, Iss, Pp-(2020)
Molecular Genetics and Metabolism Reports

مصطلحات موضوعية: Microdialysis, h.a.d., hours after dose, t½, terminal half-life, CNS distribution, Pharmacology, Cisterna magna, CSF, cerebrospinal fluid, Sulfamidase, Endocrinology, Cerebrospinal fluid, MPS IIIA, mucopolysaccharidosis type III A, Mucopolysaccharidosis IIIA, MSD-ECL, meso scale discovery electrochemiluminescence, lcsh:QH301-705.5, V, volume of distribution, education.field_of_study, lcsh:R5-920, Chemistry, Enzyme replacement therapy, aCSF, artificial cerebrospinal fluid, medicine.anatomical_structure, lcsh:Medicine (General), Research Paper, CM-rhSulfamidase, chemically modified recombinant human sulfamidase, ID, identifier, rhSulfamidase, recombinant human sulfamidase, Population, HS, heparan sulfate, LLOQ, lower limit of quantification, Blood–brain barrier, CNS, central nervous system, AUClast, area under the concentration-time curve from t = 0 to the last observed concentration, M6PR, mannose-6-phosphate receptor, Pharmacokinetics, PBS, phosphate buffered saline, Interstitial fluid, i.v., intravenous, Genetics, medicine, Cmax, maximum concentration, education, Molecular Biology, AUC∞, area under the concentration-time curve from t = 0 to infinity, SGSH, N-sulfoglucosamine sulfohydrolase, lcsh:Biology (General), IF, interstitial fluid, PK, pharmacokinetics, SD, standard deviation, CL, clearance

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ea5926859a8eba56927ab317fa4978ed
http://www.sciencedirect.com/science/article/pii/S221442691930196X

المؤلفون: Hideto Morimoto, Kazuki Miyauchi, Atsushi Imakiire, Satowa Tanaka, Jun Ito, Kohtaro Minami, Shinji Kakimoto, Hiroyuki Sonoda, Sachiho Kida, Tohru Hirato, Yuri Koshimura

المصدر: Molecular Genetics and Metabolism. 132:S105-S106

مصطلحات موضوعية: Pathology, medicine.medical_specialty, business.industry, Endocrinology, Diabetes and Metabolism, Blood–brain barrier, Biochemistry, Endocrinology, N-Sulfoglucosamine sulfohydrolase, medicine.anatomical_structure, Non clinical, Genetics, medicine, business, Molecular Biology, Mucopolysaccharidosis Type IIIA

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_________::12111ad8b3ec69a33bd12e2d47d426eb
https://doi.org/10.1016/j.ymgme.2020.12.258

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المؤلفون: Adeline A. Lau, John J. Hopwood, Lauren S. Whyte, Kim M. Hemsley

المصدر: Molecular Genetics and Metabolism Reports, Vol 5, Iss C, Pp 60-62 (2015)

مصطلحات موضوعية: lcsh:R5-920, biology, Chemistry, Mucopolysaccharidosis, Disease pathogenesis, medicine.disease, Enzyme assay, N-sulfoglucosamine sulfohydrolase, Endocrinology, N-Sulfoglucosamine sulfohydrolase, Activity measurements, Biochemistry, lcsh:Biology (General), Genetics, biology.protein, medicine, Fluorometry, Enzyme activity, lcsh:Medicine (General), Molecular Biology, lcsh:QH301-705.5, Sanfilippo

URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b149468e3d565d7ed16f840bc0e7f375

المؤلفون: Lauren S, Whyte, John J, Hopwood, Kim M, Hemsley, Adeline A, Lau

المصدر: Molecular Genetics and Metabolism Reports

مصطلحات موضوعية: N-sulfoglucosamine sulfohydrolase, Short Communication, Fluorometry, Enzyme activity, Sanfilippo, Mucopolysaccharidosis

URL الوصول: https://explore.openaire.eu/search/publication?articleId=pmid________::6ded4dcc975fb4ff590704494f166dcc
https://pubmed.ncbi.nlm.nih.gov/28652977

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