المؤلفون: Young Bae Sohn, Raymond Wang, Jane Ashworth, Pierre Broqua, Mireille Tallandier, Jean-Louis Abitbol, Erin Jozwiak, Laura Pollard, Timothy C. Wood, Tariq Aslam, Paul R. Harmatz

المصدر: Molecular Genetics and Metabolism Reports, Vol 38, Iss , Pp 101041- (2024)

مصطلحات موضوعية: Mucopolysaccharidosis type VI, Leukocyte GAG, Skin GAG, Corneal opacification measurements, Carotid intima media thickness, Medicine (General), R5-920, Biology (General), QH301-705.5

وصف الملف: electronic resource

العلاقة: http://www.sciencedirect.com/science/article/pii/S2214426923000873Test; https://doaj.org/toc/2214-4269Test

الوصول الحر: https://doaj.org/article/efb44fff9b354619a473658aafb0ccd6Test

المؤلفون: Pascale Tuyaa-Boustugue, Ingrid Jantzen, Haoyue Zhang, Sarah P. Young, Pierre Broqua, Mireille Tallandier, Eugeni Entchev

المصدر: Molecular Genetics and Metabolism Reports, Vol 37, Iss , Pp 101011- (2023)

مصطلحات موضوعية: Mucopolysaccharidosis type VI, Mucopolysaccharidosis type I, Lysosome, Odiparcil, MPS VI therapy, MPS I therapy, Medicine (General), R5-920, Biology (General), QH301-705.5

وصف الملف: electronic resource

العلاقة: http://www.sciencedirect.com/science/article/pii/S2214426923000575Test; https://doaj.org/toc/2214-4269Test

الوصول الحر: https://doaj.org/article/39015b4be7194098910689d76cf2f4c1Test

المؤلفون: Eugeni Entchev, Ingrid Jantzen, Philippe Masson, Stephanie Bocart, Bruno Bournique, Jean-Michel Luccarini, Andre Bouchot, Olivier Lacombe, Jean-Louis Junien, Pierre Broqua, Mireille Tallandier

المصدر: PLoS ONE, Vol 15, Iss 5, p e0233032 (2020)

مصطلحات موضوعية: Medicine, Science

وصف الملف: electronic resource

العلاقة: https://doaj.org/toc/1932-6203Test

الوصول الحر: https://doaj.org/article/18aaeaa92fd84ae2983028dab3ec1275Test

المؤلفون: Nathalie Guffon, Pratima Chowdary, Elisa Leão Teles, Derralynn Hughes, Julia B. Hennermann, Philippe Huot‐Marchand, Elodie Faudot‐Vernier, Olivier Lacombe, Anne Fiquet, Marie‐Paule Richard, Jean‐Louis Abitbol, Mireille Tallandier, Christian J. Hendriksz

المصدر: Journal of Inherited Metabolic Disease. 45:340-352

مصطلحات موضوعية: Mucopolysaccharidosis VI, N-Acetylgalactosamine-4-Sulfatase, Genetics, Humans, Enzyme Replacement Therapy, Glycosides, Mucopolysaccharidoses, Genetics (clinical), Glycosaminoglycans

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a52fa76f7d92f85e65eee712b97b6256Test
https://doi.org/10.1002/jimd.12467Test

المؤلفون: Olivier Lacombe, Haoyue Zhang, Aurelie Roussey, Ingrid Jantzen, Laurence Feraille, Eugeni Entchev, Jeanne-Marie Germain, Nicolas Cimbolini, Sophie Antonelli, Virginie Mauro, Jean-Michel Luccarrini, Sarah P. Young, Mireille Tallandier

المصدر: Molecular genetics and metabolism. 135(2)

مصطلحات موضوعية: Arylsulfatase B, Pathology, medicine.medical_specialty, Eye Diseases, N-Acetylgalactosamine-4-Sulfatase, Endocrinology, Diabetes and Metabolism, Mucopolysaccharidosis type VI, Eye, Biochemistry, Glycosaminoglycan, Mice, Endocrinology, Cornea, Genetics, medicine, Lysosomal storage disease, Animals, Humans, Glycosides, Molecular Biology, Corneal epithelium, Mucopolysaccharidosis VI, Chemistry, Enzyme replacement therapy, medicine.disease, eye diseases, Disease Models, Animal, medicine.anatomical_structure, Phenotype, Eye disorder, sense organs

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::124491920ae9a33c3b046ad400f72584Test
https://pubmed.ncbi.nlm.nih.gov/34417096Test

المؤلفون: Bruno Bournique, Eugeni Entchev, Stephanie Bocart, Ingrid Jantzen, Jean-Louis Junien, Jean-Michel Luccarini, Olivier Lacombe, Philippe Masson, Mireille Tallandier, Pierre Broqua, André Bouchot

المساهمون: Inventiva Pharma [Daix], Dispositif Inter-régional d'Imagerie Cellulaire [Dijon] (DImaCell), Procédés Alimentaires et Microbiologiques (PAM), Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ingénierie et biologie cellulaire et tissulaire (IBCT (ex IFR133)), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté])-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté])-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement, This study is funded by Inventiva SA ('Inventiva')., Bodescot, Myriam, Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Agroécologie [Dijon], Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)

المصدر: PLoS ONE
PLoS ONE, Public Library of Science, 2020, 15 (5), pp.e0233032. ⟨10.1371/journal.pone.0233032⟩
PLoS ONE, Vol 15, Iss 5, p e0233032 (2020)
PLoS ONE, 2020, 15 (5), pp.e0233032. ⟨10.1371/journal.pone.0233032⟩

مصطلحات موضوعية: 0301 basic medicine, Male, Mucopolysaccharidosis type VI, Respiratory System, Administration, Oral, Glycosaminoglycan, Rats, Sprague-Dawley, White Blood Cells, Mice, 0302 clinical medicine, Oral administration, Animal Cells, Medicine and Health Sciences, Glycosides, Cells, Cultured, Connective Tissue Cells, Glycosaminoglycans, Multidisciplinary, Mucopolysaccharidosis VI, Chemistry, Chondroitin Sulfates, Animal Models, 3. Good health, Trachea, medicine.anatomical_structure, Experimental Organism Systems, Connective Tissue, [SDV.SP.PHARMA] Life Sciences [q-bio]/Pharmaceutical sciences/Pharmacology, Medicine, Female, Biological Cultures, Cellular Types, Anatomy, Cellular Structures and Organelles, Research Article, medicine.medical_specialty, Immune Cells, Science, Immunology, Dermatan Sulfate, Mouse Models, In Vitro Techniques, Research and Analysis Methods, 03 medical and health sciences, Model Organisms, In vivo, Internal medicine, medicine, Animals, Humans, Blood Cells, Cartilage, Biology and Life Sciences, Endothelial Cells, Kidneys, Cell Biology, Renal System, Fibroblasts, Cell Cultures, In vitro, Mice, Mutant Strains, Rats, Mice, Inbred C57BL, Disease Models, Animal, 030104 developmental biology, Endocrinology, Biological Tissue, Cell culture, Animal Studies, [SDV.SP.PHARMA]Life Sciences [q-bio]/Pharmaceutical sciences/Pharmacology, Cattle, Lysosomes, 030217 neurology & neurosurgery

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f57cf8f55479c409e7f909dd02436ef8Test
https://www.hal.inserm.fr/inserm-02894692Test

المؤلفون: Ashlee R. Stiles, Eugeni Entchev, Olivier Lacombe, Deeksha Bali, Sarah P. Young, Mireille Tallandier, Haoyue Zhang

المصدر: Molecular Genetics and Metabolism. 129:S165

مصطلحات موضوعية: chemistry.chemical_compound, Endocrinology, Chromatography, chemistry, Endocrinology, Diabetes and Metabolism, Mucopolysaccharidosis type VI, Genetics, Uplc ms ms, Chondroitin sulfate, Molecular Biology, Biochemistry, Dermatan sulfate

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_________::933544ecc3687472ca356476fd9b53c9Test
https://doi.org/10.1016/j.ymgme.2019.11.440Test

المؤلفون: Jean-Louis Junien, Xavier Gawronski, Laurence Feraille, Ingrid Jantzen, Eugeni Entchev, Jean-Louis Abitbol, Mireille Tallandier, Jean-Michel Luccarini, Pierre Broqua

المصدر: Molecular Genetics and Metabolism. 123:S42-S43

مصطلحات موضوعية: 0301 basic medicine, business.industry, Endocrinology, Diabetes and Metabolism, Biochemistry, 03 medical and health sciences, 030104 developmental biology, Endocrinology, Murine model, Genetics, Cancer research, Medicine, Substrate reduction therapy, business, Molecular Biology

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_________::338e119d30cf7b89dc4b317c9bbaf570Test
https://doi.org/10.1016/j.ymgme.2017.12.093Test

المؤلفون: Luc Rochette, Stéphanie Sueur, Matthieu Pesant, Mireille Tallandier, Paul A. Grimaldi, Jean-Louis Connat, Patrick Dutartre

المصدر: Cardiovascular Research. 69:440-449

مصطلحات موضوعية: Programmed cell death, medicine.medical_specialty, Physiology, Blotting, Western, Peroxisome proliferator-activated receptor, Apoptosis, Caspase 3, DNA Fragmentation, Biology, Transfection, medicine.disease_cause, Cell Line, GW501516, Physiology (medical), Internal medicine, In Situ Nick-End Labeling, medicine, Animals, PPAR delta, Viability assay, Receptor, chemistry.chemical_classification, Reverse Transcriptase Polymerase Chain Reaction, Hydrogen Peroxide, Catalase, medicine.disease, Rats, Up-Regulation, Cell biology, Oxidative Stress, Thiazoles, Endocrinology, chemistry, Caspases, Cardiology and Cardiovascular Medicine, Myoblasts, Cardiac, Oxidative stress

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::22ae44ad9a248754c9ea40bc88de9cb6Test
https://doi.org/10.1016/j.cardiores.2005.10.019Test

المؤلفون: Jean-Louis Junien, Ingrid Jantzen, Eugeni Entchev, Sébastien Jacquet, Olivier Lacombe, Jean-Louis Abitbol, Philippe Masson, Pierre Broqua, Mireille Tallandier, Véronique Douet

المصدر: Molecular Genetics and Metabolism. 120:S129

مصطلحات موضوعية: Endocrinology, In vivo, Chemistry, Endocrinology, Diabetes and Metabolism, Mucopolysaccharidosis, Genetics, medicine, Substrate reduction therapy, Pharmacology, medicine.disease, Molecular Biology, Biochemistry, In vitro

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_________::19dab0f4c8dc815d365c5aafedaf0c7aTest
https://doi.org/10.1016/j.ymgme.2016.11.339Test