المؤلفون: Alexander Giaramita, Nathan Wise, Jon Vincelette, Rolando De Angelis, Brett E. Crawford, Sherry Bullens, Nicole Galicia, Melanie J. Lo, Jeremy L. Van Vleet, Sushmita M Roy, Gouri Yogalingam, Amanda R. Luu, Linley Mangini, Alessandra d'Azzo, Vishal Agrawal, Glenn Pacheco, Sylvia Fong, Jonathan H. LeBowitz, Terri Christianson, Hio Wong, Roger Lawrence, Britta Handyside, Harry J Sterling, Stuart Bunting, Chuck Hague, Joseph C Chen

المصدر: The Journal of Biological Chemistry

مصطلحات موضوعية: 0301 basic medicine, safety, medicine.medical_specialty, GM1 gangliosidosis, Biochemistry, enzyme replacement therapy (ERT), 03 medical and health sciences, Mice, Internal medicine, Lysosome, biophysics, Lysosomal storage disease, medicine, Animals, cation-independent mannose-6-phosphate receptor, Enzyme Replacement Therapy, unfolded protein response (UPR), Molecular Biology, Ganglioside, Gangliosidosis, GM1, 030102 biochemistry & molecular biology, Chemistry, Chinese hamster ovary cell, Endoplasmic reticulum, neurodegeneration, toxicity, Enzyme replacement therapy, Cell Biology, medicine.disease, beta-Galactosidase, gene therapy, 030104 developmental biology, medicine.anatomical_structure, Endocrinology, GLB1, lysosomal storage disease, Unfolded protein response, lysosome, endoplasmic reticulum stress

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1842a37d379f3461db62d067305f34f9Test
http://europepmc.org/articles/PMC7521651Test

المؤلفون: Brett E. Crawford, Adam Harris, Jeremy L. Van Vleet, Roberto Giugliani, Sanjay Chandriani, Roger Lawrence, Gouri Yogalingam, Linley Mangini, Nathan T. Martin, Wyatt T. Clark, Alessandra d'Azzo, Jonathan H. LeBowitz

المصدر: Repositório Institucional da UFRGS
Universidade Federal do Rio Grande do Sul (UFRGS)
instacron:UFRGS
Molecular Genetics and Metabolism Reports
Molecular Genetics and Metabolism Reports, Vol 21, Iss, Pp-(2019)

مصطلحات موضوعية: Glycoconjugate, MPS, mucopolysaccharidosis, GRIL-LC/MS, glycan reductive isotope labeling liquid chromatography mass spectrometry, BMP, Bis(monoacylglycero) phosphate, TIC, total ion current, chemistry.chemical_compound, 0302 clinical medicine, Endocrinology, KS, keratan sulfate, Disease biomarkers, Beta-galactosidase, Hex, hexose, lcsh:QH301-705.5, chemistry.chemical_classification, lcsh:R5-920, 0303 health sciences, Gangliosidose GM1, biology, 030305 genetics & heredity, GlcNAc, N-acetylglucosamine, GLB1, Biochemistry, HexNAc, N-acetylhexosamine, lcsh:Medicine (General), Research Paper, Glycan, GM1 gangliosidosis, GLB1, β-galactosidase, A2G2, Oxford glycan naming designation for NA2 glycan, 03 medical and health sciences, Glycoanalysis, Glycolipid, dp, degree of polymerization, Genetics, Glycan metabolites, Molecular Biology, NRE, non-reducing end, m/z, mass over charge, Ganglioside, Man, mannose, carbohydrates (lipids), Gal, galactose, Enzyme, Biomarcadores, lcsh:Biology (General), chemistry, Galactose, biology.protein, XIC, extracted ion current, 030217 neurology & neurosurgery

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fad1e65eec41e62a514a14a12ceec192Test