المؤلفون: Sheela Sitaraman, Richard Roxburgh, Kristina Gutschmidt, Ela Stefanescu, Drago Bratkovic, Thomas Burrow, Kornblum Cornelia, Kristl Claeys, Miriam Freimer, Ozlem Goker-Alpan, Srilakshmi Kuchipudi, Alan Pestronk, Wolfgang Löscher, Francoise Bouhour, Maria Judit Molnar, Ans T. van der Ploeg, Halina Bartosik-Psujek, Mitchell Goldman, Robert D. Henderson, Stephanie Dearmey, Colin Quinn, Paula R. Clemens, Priya S. Kishnani, Jennifer B Avelar, Nicola Longo, Shahram Attarian, Robert Hopkin, Tomo Sawada, Blaž Koritnik, George Konstantinos Papadimas, Hideaki Shiraishi, Christopher Lindberg, Jin-Hong Shin, Ivaylo Tarnev, Tahseen Mozaffar, Heather Lau, Michel Tchan, Jozsef Janszky, Tobias Ruck, Sabrina Sacconi, Benedikt Schoser, Hashiguchi Akihiro, Patrick Deegan, Ernest Butler, Nuria Vidal-Fernandez, Antonio Toscano, Tarekegn Hiwot, Gee Kim, Emmanuelle Salort-Campana, Jeff Castelli, Pascal Laforet, Céline Tard, Crystal Eldridge, Aneal Khan, Stephan Wenninger, Simona Fecarotta, Jordi Díaz-Manera, Jorge Alonso-Pérez, Yin-Hsiu Chien, Mark Tarnopolsky, Olimpia Musumeci, Hiroshi Kobayashi, Helio Pedro, Jonathan Cauci, Agnes Sebok, Cynthia Bodkin, Hai Jiang, Julie Berthy, Vescei Laszlo, Derralynn Hughes, David Reyes-Leiva, Aleksandra Dominovic-Kovacevic, Mazen M. Dimachkie, Hernan Amartino, Hani Kushlaf, Barry J. Byrne, Giancarlo Parenti, Henning Andersen, Mark Roberts, Marie Wencel, Jaime Vengoechea

المساهمون: Schoser, B., Roberts, M., Byrne, B. J., Sitaraman, S., Jiang, H., Laforet, P., Toscano, A., Castelli, J., Diaz-Manera, J., Goldman, M., van der Ploeg, A. T., Bratkovic, D., Kuchipudi, S., Mozaffar, T., Kishnani, P. S., Sebok, A., Pestronk, A., Dominovic-Kovacevic, A., Khan, A., Koritnik, B., Tard, C., Lindberg, C., Quinn, C., Eldridge, C., Bodkin, C., Reyes-Leiva, D., Hughes, D., Stefanescu, E., SALORT-CAMPANA, E., Butler, E., Bouhour, F., Kim, G., Konstantinos Papadimas, G., Parenti, G., Bartosik-Psujek, H., Kushlaf, H., Akihiro, H., Lau, H., Pedro, H., Andersen, H., Amartino, H., Shiraishi, H., Kobayashi, H., Tarnev, I., Vengoechea, J., Avelar, J., Shin, J. -H., Cauci, J., Alonso-Perez, J., Janszky, J., Berthy, J., Cornelia, K., Gutschmidt, K., Claeys, K., Judit Molnar, M., Wencel, M., Tarnopolsky, M., Dimachkie, M., Tchan, M., Freimer, M., Longo, N., Vidal-Fernandez, N., Musumeci, O., Goker-Alpan, O., Deegan, P., Clemens, P. R., Roxburgh, R., Henderson, R., Hopkin, R., Sacconi, S., Fecarotta, S., Attarian, S., Wenninger, S., Dearmey, S., Hiwot, T., Burrow, T., Ruck, T., Sawada, T., Laszlo, V., Loscher, W., Chien, Y. -H., Pediatrics

المصدر: LANCET NEUROLOGY
r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
instname
The Lancet Neurology, 20(12), 1027-1037. Lancet Publishing Group
PROPEL Study Group 2021, ' Safety and efficacy of cipaglucosidase alfa plus miglustat versus alglucosidase alfa plus placebo in late-onset Pompe disease (PROPEL) : an international, randomised, double-blind, parallel-group, phase 3 trial ', The Lancet Neurology, vol. 20, no. 12, pp. 1027-1037 . https://doi.org/10.1016/S1474-4422Test(21)00331-8

مصطلحات موضوعية: education.field_of_study, medicine.medical_specialty, 1-Deoxynojirimycin, Adolescent, Glycogen Storage Disease Type II, business.industry, Population, alpha-Glucosidases, Enzyme replacement therapy, Placebo, Treatment Outcome, Double-Blind Method, SDG 3 - Good Health and Well-being, Internal medicine, Miglustat, medicine, Clinical endpoint, Humans, Respiratory function, Neurology (clinical), Adverse effect, education, business, Alglucosidase alfa, medicine.drug

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7ca8d17d8a65ecd5fd078c358714459bTest
https://doi.org/10.1016/s1474-4422Test(21)00331-8

المؤلفون: Sandra Strollo, Gianfranco Peluso, Caterina Porto, Gerlind Sulzenbacher, Carla Damiano, Nadia Minopoli, Antonietta Tarallo, Marco Moracci, Véronique Roig-Zamboni, Giancarlo Parenti, Beatrice Cobucci-Ponzano, Maria Carmina Ferrara, Roberta Iacono

المساهمون: Department of Biology, University of Naples 'Federico II', Architecture et fonction des macromolécules biologiques (AFMB), Centre National de la Recherche Scientifique (CNRS)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Aix Marseille Université (AMU), Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), University of Naples Federico II = Università degli studi di Napoli Federico II

المصدر: Journal of Enzyme Inhibition and Medicinal Chemistry
article-version (VoR) Version of Record
Journal of enzyme inhibition and medicinal chemistry
36 (2021): 2068–2079. doi:10.1080/14756366.2021.1975694
info:cnr-pdr/source/autori:Iacono, Roberta; Minopoli, Nadia; Ferrara, Maria Carmina; Tarallo, Antonietta; Damiano, Carla; Porto, Caterina; Strollo, Sandra; Roig-Zamboni, Veronique; Peluso, Gianfranco; Sulzenbacher, Gerlind; Cobucci-Ponzano, Beatrice; Parenti, Giancarlo; Moracci, Marco/titolo:Carnitine is a pharmacological allosteric chaperone of the human lysosomal alpha-glucosidase/doi:10.1080%2F14756366.2021.1975694/rivista:Journal of enzyme inhibition and medicinal chemistry (Print)/anno:2021/pagina_da:2068/pagina_a:2079/intervallo_pagine:2068–2079/volume:36
Journal of Enzyme Inhibition and Medicinal Chemistry, Informa Healthcare, 2021, 36 (1), pp.2068-2079. ⟨10.1080/14756366.2021.1975694⟩
Journal of Enzyme Inhibition and Medicinal Chemistry, Vol 36, Iss 1, Pp 2068-2079 (2021)
Journal of Enzyme Inhibition and Medicinal Chemistry, 2021, 36 (1), pp.2068-2079. ⟨10.1080/14756366.2021.1975694⟩

مصطلحات موضوعية: glycogen storage disease type 2, [SDV]Life Sciences [q-bio], Allosteric regulation, RM1-950, Pharmacology, alpha-Glucosidase, 03 medical and health sciences, Structure-Activity Relationship, 0302 clinical medicine, Allosteric Regulation, Carnitine, Drug Discovery, medicine, Humans, Glycoside Hydrolase Inhibitors, 030304 developmental biology, chemistry.chemical_classification, 0303 health sciences, biology, Dose-Response Relationship, Drug, Molecular Structure, Chemistry, carbohydrate active enzymes, Metabolic disorder, lysosomal disease, alpha-Glucosidases, General Medicine, Enzyme replacement therapy, medicine.disease, Small molecule, 3. Good health, Pharmacological chaperone, Enzyme, α-Glucosidase, orphan drugs, Chaperone (protein), biology.protein, Therapeutics. Pharmacology, Lysosomes, 030217 neurology & neurosurgery, medicine.drug, Research Article, Research Paper, Molecular Chaperones

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2cccde6b8d490ed35096955d6bfb78efTest
http://europepmc.org/articles/PMC8477953Test

المؤلفون: Vincenza Gragnaniello, Federica Deodato, Serena Gasperini, Maria Alice Donati, Clementina Canessa, Simona Fecarotta, Antonia Pascarella, Giuseppe Spadaro, Daniela Concolino, Alberto Burlina, Giancarlo Parenti, Pietro Strisciuglio, Agata Fiumara, Roberto Della Casa

المساهمون: Gragnaniello, Vincenza, Deodato, Federica, Gasperini, Serena, Alice Donati, Maria, Canessa, Clementina, Fecarotta, Simona, Pascarella, Antonia, Spadaro, Giuseppe, Concolino, Daniela, Burlina, Alberto, Parenti, Giancarlo, Strisciuglio, Pietro, Fiumara, Agata, DELLA CASA, Roberto

المصدر: Italian journal of pediatrics. 48(1)

مصطلحات موضوعية: Italy, Glycogen Storage Disease Type II, Immunity, Pompe disease, Humans, CRIM status, Desensitization, Immune tolerance induction, Infusion associated reactions, Enzyme Replacement Therapy, alpha-Glucosidases, Child

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f88fee145192a0a9cdc14ae65a1fafacTest
https://pubmed.ncbi.nlm.nih.gov/35248118Test

المؤلفون: Marcella Coletta, Daria Maria Monti, Carla Damiano, Sandra Strollo, Roman S. Polishchuk, Alessia Indrieri, Roberta Iacono, Francesca Zappa, Maria Antonietta De Matteis, Elena Polishchuk, Giancarlo Parenti, Edoardo Nusco, Simona Fecarotta, Nadia Minopoli, Diego L. Medina, Caterina Porto, Antonietta Tarallo, Marco Moracci, Paola Imbimbo

المساهمون: Tarallo, A., Damiano, C., Strollo, S., Minopoli, N., Indrieri, A., Polishchuk, E., Zappa, F., Nusco, E., Fecarotta, S., Porto, C., Coletta, M., Iacono, R., Moracci, M., Polishchuk, R., Medina, D. L., Imbimbo, P., Monti, D. M., De Matteis, M. A., Parenti, G.

المصدر: EMBO Molecular Medicine
EMBO Molecular Medicine, Vol 13, Iss 11, Pp n/a-n/a (2021)

مصطلحات موضوعية: Medicine (General), Metabolic myopathy, QH426-470, Pharmacology, Resveratrol, medicine.disease_cause, Article, chemistry.chemical_compound, Mice, R5-920, Genetics, Edaravone, medicine, Idebenone, Animals, Humans, alpha-glucosidase, alpha‐glucosidase, N‐acetylcysteine, Musculoskeletal System, oxidative stre, Glycogen, business.industry, Glycogen Storage Disease Type II, Autophagy, Pompe disease, alpha-Glucosidases, Enzyme replacement therapy, Articles, medicine.disease, N-acetylcysteine, Oxidative Stress, Metabolism, chemistry, Molecular Medicine, Genetics, Gene Therapy & Genetic Disease, business, Oxidative stress, medicine.drug, enzyme replacement therapy

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4b1dad04385c6e65d0e7d15fc637b04bTest
http://hdl.handle.net/11588/861128Test

المؤلفون: Maria Rosaria Magaldi, Luigi Michele Pavone, Emma Acampora, Simona Fecarotta, Giovanni Esposito, Francesca Iacobellis, Marta Rubino, Salvatore Esposito, Paolo Calabrò, Salvatore Cappabianca, Antonietta Tarallo, Emanuele Monda, Marcella Sasso, Martina Caiazza, Maria Paola Belfiore, Roberto Grassi, Valeria De Pasquale, Giancarlo Parenti, Antonio Pisani, Giuseppe Limongelli

المساهمون: Belfiore, M. P., Iacobellis, F., Acampora, E., Caiazza, M., Rubino, M., Monda, E., Magaldi, M. R., Tarallo, A., Sasso, M., De Pasquale, V., Grassi, R., Cappabianca, S., Calabro, P., Fecarotta, S., Esposito, S., Esposito, G., Pisani, A., Pavone, L. M., Parenti, G., Limongelli, G., Belfiore, Maria Paola, Iacobellis, Francesca, Acampora, Emma, Caiazza, Martina, Rubino, Marta, Monda, Emanuele, Magaldi, Maria Rosaria, Tarallo, Antonietta, Sasso, Marcella, De Pasquale, Valeria, Grassi, Roberto, Cappabianca, Salvatore, Calabrò, Paolo, Fecarotta, Simona, Esposito, Salvatore, Esposito, Giovanni, Pisani, Antonio, Pavone, Luigi Michele, Parenti, Giancarlo, Limongelli, Giuseppe

المصدر: PLoS ONE
PLoS ONE, Vol 15, Iss 5, p e0233050 (2020)

مصطلحات موضوعية: 0301 basic medicine, Aortic valve, Aortic arch, Male, Pathology, Mucopolysaccharidosis, 030204 cardiovascular system & hematology, Mice, Mucopolysaccharidosis III, 0302 clinical medicine, Aortic sinus, Medicine and Health Sciences, Group-Specific Staining, Aorta, Mice, Knockout, Staining, Multidisciplinary, Glycogen Storage Disease Type II, Heart, Animal Models, medicine.anatomical_structure, Experimental Organism Systems, Echocardiography, Genetic Diseases, Descending aorta, Aortic Valve, cardiovascular system, Medicine, Anatomy, Cellular Structures and Organelles, Research Article, medicine.medical_specialty, Science, Aortic Diseases, Mouse Models, Research and Analysis Methods, 03 medical and health sciences, Model Organisms, Autosomal Recessive Diseases, medicine.artery, Ascending aorta, Acetylglucosaminidase, medicine, Animals, Animal Models of Disease, Clinical Genetics, business.industry, Hematoxylin Staining, Biology and Life Sciences, alpha-Glucosidases, Cell Biology, Mucopolysaccharidoses, medicine.disease, Fabry disease, Lysosomal Storage Diseases, Disease Models, Animal, 030104 developmental biology, Specimen Preparation and Treatment, alpha-Galactosidase, Animal Studies, Cardiovascular Anatomy, Fabry Disease, Blood Vessels, business, Lysosomes

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0541509135cf1c7e4fd7ea84fa8482dcTest
https://pubmed.ncbi.nlm.nih.gov/32428018Test

المؤلفون: A Catzola, Giancarlo Parenti, Giuseppe Spadaro, Antonio Pecoraro, V. Gragnaniello, Antonietta Tarallo, R. Della Casa, Simona Fecarotta

المساهمون: Gragnaniello, V., Fecarotta, S., Pecoraro, A., Tarallo, A., Catzola, Andrea, Spadaro, G., Parenti, G., Della Casa, R.

المصدر: Neurological Sciences. 40:1453-1455

مصطلحات موضوعية: Male, Pediatrics, medicine.medical_specialty, Hormone Replacement Therapy, Acid alpha-glucosidase, medicine.medical_treatment, Cardiomyopathy, Associated reaction, Desensitization, Dermatology, Metabolic myopathy, 03 medical and health sciences, 0302 clinical medicine, Humans, Medicine, 030212 general & internal medicine, Infusion, Alglucosidase alfa, Desensitization (medicine), Glycogen Storage Disease Type II, business.industry, Pompe disease, Infant, alpha-Glucosidases, General Medicine, Enzyme replacement therapy, medicine.disease, Psychiatry and Mental health, Desensitization, Immunologic, Premedication, Neurology (clinical), business, 030217 neurology & neurosurgery, Tranexamic acid, medicine.drug

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::88dae1b5d9cf0886b5fac4e39e05ad3aTest
https://doi.org/10.1007/s10072-019-03744-3Test

المؤلفون: Daniele D'Alonzo, Annalisa Guaragna, Giancarlo Parenti, Frances M. Platt, Roberta Iacono, Beatrice Cobucci-Ponzano, Caterina Porto, Mylene Huebecker, Giovanni Palumbo, David A. Priestman, Marco Moracci, Maria De Fenza

المساهمون: D'Alonzo, Daniele, De Fenza, Maria, Porto, Caterina, Iacono, Roberta, Huebecker, Mylene, Cobucci-Ponzano, Beatrice, Priestman, David A, Platt, France, Parenti, Giancarlo, Moracci, Marco, Palumbo, Giovanni, Guaragna, Annalisa

المصدر: Journal of medicinal chemistry 60 (2017): 9462–9469. doi:10.1021/acs.jmedchem.7b00646
info:cnr-pdr/source/autori:D'Alonzo D, De Fenza M, Porto C, Iacono R, Huebecker M, Cobucci-Ponzano B, Priestman D, Platt FM, Parenti G, Moracci M, Palumbo G, Guaragna A./titolo:N-Butyl-L-Deoxynojirimycin (L-NBDNJ): Synthesis of an Allosteric Enhancer of alpha-Glucosidase Activity for the Treatment of Pompe Disease./doi:10.1021%2Facs.jmedchem.7b00646/rivista:Journal of medicinal chemistry/anno:2017/pagina_da:9462/pagina_a:9469/intervallo_pagine:9462–9469/volume:60

مصطلحات موضوعية: Models, Molecular, 0301 basic medicine, 1-Deoxynojirimycin, Allosteric regulation, Iminosugar, Stereoisomerism, de novo synthesis, 01 natural sciences, Cell Line, law.invention, 03 medical and health sciences, Allosteric Regulation, law, Drug Discovery, Miglustat, iminosugars, medicine, Humans, Enzyme Inhibitors, Glycogen Storage Disease Type II, 010405 organic chemistry, Chemistry, Pompe disease, alpha-Glucosidases, Fibroblasts, acid alpha-glucosidase, pharmacological chaperone, 0104 chemical sciences, Enzyme Activation, De novo synthesis, 030104 developmental biology, Biochemistry, Cell culture, Recombinant DNA, Molecular Medicine, Enantiomer, Lysosomes, pompe disease, GAA, iminosugars, NBDNJ, medicine.drug

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::99dfd2d5c41188a702d043f6a2f589a6Test
https://doi.org/10.1021/acs.jmedchem.7b00646Test

المؤلفون: Stijn L. M. in ‘t Groen, Gerben J. Schaaf, Tom J.M. van Gestel, Giancarlo Parenti, Antonietta Tarallo, Ans T. van der Ploeg, Björn Boomaars, Monica Cardone, W.W.M. Pim Pijnappel, Bart de Jong

المساهمون: Clinical Genetics, Pediatrics, Schaaf, Gerben J., van Gestel, Tom J M, In 't Groen, Stijn L M, de Jong, Bart, Boomaars, Björn, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, van der Ploeg, Ans T., Pijnappel, W W M Pim

المصدر: Acta neuropathologica communications (online), 6:119. BioMed Central Ltd.
Acta Neuropathologica Communications, Vol 6, Iss 1, Pp 1-16 (2018)
Acta Neuropathologica Communications

مصطلحات موضوعية: Male, 0301 basic medicine, Barium Compounds, Cell, Endogeny, Lysosomal storage disease, lcsh:RC346-429, Mice, Satellite cells, Muscle regeneration, Glycogen Storage Disease Type II, Age Factors, PAX7 Transcription Factor, Pompe disease, medicine.anatomical_structure, Acid alpha-glucosidase, Female, Stem cell, Cell activation, Glycogen, medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, Satellite Cells, Skeletal Muscle, Mice, Transgenic, Glycogenosis type II, Metabolic myopathy, Biology, Cardiotoxins, Pathology and Forensic Medicine, 03 medical and health sciences, Cellular and Molecular Neuroscience, Chlorides, Lysosomal-Associated Membrane Protein 1, Internal medicine, medicine, Animals, Regeneration, Muscle, Skeletal, lcsh:Neurology. Diseases of the nervous system, Research, Regeneration (biology), nutritional and metabolic diseases, alpha-Glucosidases, medicine.disease, Mice, Inbred C57BL, Disease Models, Animal, Ki-67 Antigen, 030104 developmental biology, Endocrinology, Laminin, Neurology (clinical), Satellite cell

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1029f9824de9c71c4cfcf804a71a456aTest
https://pure.eur.nl/en/publications/05c12ca5-d6c3-4af4-94ef-90ee0d6c9442Test

المؤلفون: Véronique Roig-Zamboni, Beatrice Cobucci-Ponzano, Roberta Iacono, Maria Carmina Ferrara, Stanley Germany, Yves Bourne, Giancarlo Parenti, Marco Moracci, Gerlind Sulzenbacher

المساهمون: Architecture et fonction des macromolécules biologiques (AFMB), Institut National de la Recherche Agronomique (INRA)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Università degli studi di Napoli Federico II, Architecture et fonction des macromolécules biologiques ( AFMB ), Centre National de la Recherche Scientifique ( CNRS ) -Aix Marseille Université ( AMU ) -Institut National de la Recherche Agronomique ( INRA ), Roig Zamboni, Veronique, Cobucci Ponzano, Beatrice, Iacono, Roberta, Ferrara, MARIA CARMINA, Germany, Stanley, Bourne, Yve, Parenti, Giancarlo, Moracci, Marco, Sulzenbacher, Gerlind, Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU)-Institut National de la Recherche Agronomique (INRA), University of Naples Federico II = Università degli studi di Napoli Federico II

المصدر: Nature Communications
Nature Communications, Nature Publishing Group, 2017, 8, pp.1111. ⟨10.1038/s41467-017-01263-3⟩
'Nature Communications ', vol: 8, pages: 1111-1-1111-10 (2017)
Nature Communications, Nature Publishing Group, 2017, 8, pp.1111. 〈10.1038/s41467-017-01263-3〉
Nature Communications, Vol 8, Iss 1, Pp 1-10 (2017)
Nature Communications, Nature Publishing Group, 2017, ⟨10.1038/s41467-017-01263-3⟩
Nature communications 8 (2017). doi:10.1038/s41467-017-01263-3
info:cnr-pdr/source/autori:Roig-Zamboni V.; Cobucci-Ponzano B.; Iacono R.; Ferrara M.C.; Germany S.; Bourne Y.; Parenti G.; Moracci M.; Sulzenbacher G./titolo:Structure of human lysosomal acid alpha-glucosidase-A guide for the treatment of Pompe disease/doi:10.1038%2Fs41467-017-01263-3/rivista:Nature communications/anno:2017/pagina_da:/pagina_a:/intervallo_pagine:/volume:8
Nature Communications, 2017, ⟨10.1038/s41467-017-01263-3⟩
Nature Communications, 2017, 8, pp.1111. ⟨10.1038/s41467-017-01263-3⟩

مصطلحات موضوعية: Models, Molecular, 0301 basic medicine, Protein Conformation, [SDV]Life Sciences [q-bio], Metabolic disease, General Physics and Astronomy, MESH: Catalytic Domain, MESH: Glycogen Storage Disease Type II, law.invention, MESH : Glycogen Storage Disease Type II, 0302 clinical medicine, Protein structure, MESH: Protein Conformation, law, Catalytic Domain, Lysosomal storage disease, lcsh:Science, MESH : Protein Conformation, Multidisciplinary, [SDV.BBM.BS]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Structural Biology [q-bio.BM], biology, Glycogen Storage Disease Type II, Chemistry, Chemistry (all), MESH : Catalytic Domain, 3. Good health, Cell biology, Pharmacological chaperone, [SDV.BBM.BS]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Biomolecules [q-bio.BM], 030220 oncology & carcinogenesis, MESH : alpha-Glucosidases, Recombinant DNA, MESH : Acetylcysteine, MESH: Models, Molecular, medicine.drug, MESH : Models, Molecular, Science, Allosteric regulation, MESH: alpha-Glucosidases, Drug development, Article, General Biochemistry, Genetics and Molecular Biology, Physics and Astronomy (all), 03 medical and health sciences, MESH: Acetylcysteine, Hydrolase, medicine, Humans, Gene, x-ray crystallography, Biochemistry, Genetics and Molecular Biology (all), MESH: Humans, MESH : Humans, alpha-Glucosidases, General Chemistry, MESH : Lysosomes, medicine.disease, Acetylcysteine, 030104 developmental biology, Chaperone (protein), biology.protein, lcsh:Q, Lysosomes, [ SDV.BBM.BS ] Life Sciences [q-bio]/Biochemistry, Molecular Biology/Biomolecules [q-bio.BM], MESH: Lysosomes

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5c82ffd7ffc81e6e645698e18bb52b05Test
https://hal.archives-ouvertes.fr/hal-01802847/file/s41467-017-01263-3.pdfTest

المؤلفون: Roman S. Polishchuk, Erin J. Feeney, Hossein Zare, Lishu Li, Carmine Spampanato, Monica Cardone, Fabio Annunziata, Giancarlo Parenti, Rosa Puertollano, Jeong-A Lim, Andrea Ballabio, Nina Raben

المساهمون: Spampanato, C., Feeney, E., Li, L., Cardone, M., Lim, J. A., Annunziata, Fabio, Zare, H., Polishchuk, R., Puertollano, R., Parenti, Giancarlo, Ballabio, Andrea, Raben, N.

المصدر: EMBO Molecular Medicine

مصطلحات موضوعية: Autophagosome, medicine.medical_specialty, autophagy, Genetic Vectors, Basic helix-loop-helix leucine zipper transcription factors, Vacuole, Biology, Exocytosis, Adenoviridae, 03 medical and health sciences, Mice, 0302 clinical medicine, Internal medicine, Glycogen storage disease type II, medicine, Animals, Muscle, Skeletal, Research Articles, Cells, Cultured, 030304 developmental biology, Mice, Knockout, 0303 health sciences, TFEB, Basic Helix-Loop-Helix Leucine Zipper Transcription Factors, Glycogen Storage Disease Type II, Autophagy, Pompe disease, alpha-Glucosidases, medicine.disease, acid alpha-glucosidase, 3. Good health, Cell biology, Disease Models, Animal, Endocrinology, lysosomal storage, Acid alpha-glucosidase, Molecular Medicine, Lysosomes, 030217 neurology & neurosurgery, Glycogen

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a48b84e4d50ab04e5b1ae24f9082caaeTest
http://europepmc.org/articles/PMC3662313Test