المؤلفون: Carlos A. Matos, Adriana Marcelo, Wilfred F. A. den Dunnen, Gaëtan Despres, Fatich Mechmet, Luís Pereira de Almeida, Liliana Mendonça, Nathalie Cartier, Sandra Tomé, Clévio Nóbrega, Sandro Alves, Dominique Langui, Antonin Lamaziere

المساهمون: Molecular Neuroscience and Ageing Research (MOLAR)

المصدر: Repositório Científico de Acesso Aberto de Portugal
Repositório Científico de Acesso Aberto de Portugal (RCAAP)
instacron:RCAAP
Acta Neuropathologica, 138(5), 837-858. SPRINGER
Acta Neuropathologica

مصطلحات موضوعية: 0301 basic medicine, Male, SCA patients, Purkinje cell, Gene, DOMINANT CEREBELLAR-ATAXIA, Purkinje Cells, 0302 clinical medicine, REPEAT, RAT MODEL, Mutant ataxin-3, SCA animal models, MACHADO-JOSEPH-DISEASE, ABNORMALITIES, Neurodegeneration, 24-hydroxylase, NEURODEGENERATION, Brain, Machado-Joseph Disease, Middle Aged, 3. Good health, Cell biology, CYP46A1, medicine.anatomical_structure, Cholesterol, Spinocerebellar ataxia, Rat model, Female, medicine.symptom, Abnormalities, Adult, congenital, hereditary, and neonatal diseases and abnormalities, Ataxia, Transgene, Mice, Transgenic, Nerve Tissue Proteins, Neuropathology, Biology, Repeat, Neuroprotection, Pathology and Forensic Medicine, 03 medical and health sciences, Cellular and Molecular Neuroscience, Cyp46A1, medicine, Autophagy, Cholesterol metabolism, Animals, Humans, Spinocerebellar Ataxias, medicine.disease, Dominant cerebellar-ataxia, GENE, 24-Cholesterol hydroxylase, MUTANT ATAXIN-3, Disease Models, Animal, 030104 developmental biology, 24-HYDROXYLASE, Neurology (clinical), 030217 neurology & neurosurgery

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2bd3fa96c9557159a2c2e2f365c4deebTest

المؤلفون: Annie Sittler, Sandro Alves, Alexis Brice, Martine Barkats, Béatrice Dufresnois, Jean-Gabriel Dornbierer, Alexandre Janer, Christelle Tesson, Darren P. Baker, Morwena Latouche, Merle Ruberg, Martina Marinello, Giovanni Stevanin, Khalid Hamid El Hachimi, Alice Chort

المصدر: Brain. 136:1732-1745

مصطلحات موضوعية: Adult, Cerebellum, Pathology, medicine.medical_specialty, Ataxin 7, Purkinje cell, Mice, Transgenic, Nerve Tissue Proteins, Motor Activity, Mice, Promyelocytic leukemia protein, Gene knockin, medicine, Animals, Humans, Spinocerebellar Ataxias, Gene Knock-In Techniques, Rats, Wistar, Child, Receptor, Cells, Cultured, Aged, Ataxin-7, biology, Interferon-beta, medicine.disease, Rats, Cell biology, Mice, Inbred C57BL, medicine.anatomical_structure, Ataxin, Mutation, Spinocerebellar ataxia, biology.protein, Neurology (clinical)

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3b9c34d96ef6d3b3cdd44c3bb260df1dTest
https://doi.org/10.1093/brain/awt061Test

المؤلفون: Muriel Priault, Wilfred F. A. den Dunnen, Jean-Christophe Corvol, Alexis Brice, Khalid Hamid El Hachimi, Merle Ruberg, Martine Barkats, Fanny Charbonnier-Beaupel, Annie Sittler, Marie-Paule Muriel, Thibaut Marais, Florence Cormier-Dequaire, Khadija Tahiri, Alexandra Durr, Florian Beaumatin, Danielle Seilhean, Giovanni Stevanin, Martina Marinello, Sandro Alves

المساهمون: Molecular Neuroscience and Ageing Research (MOLAR)

المصدر: Acta Neuropathologica, 128(5), 705-722. SPRINGER

مصطلحات موضوعية: Autophagosome, Male, Mice, Trinucleotide Repeats, NEURODEGENERATIVE DISEASE, GENETIC MOUSE MODELS, Cell Line, Transformed, LAMP2, MACHADO-JOSEPH-DISEASE, TOR Serine-Threonine Kinases, Microfilament Proteins, Brain, Polyglutamine tract, Lysosome, SCA7 knock-in mouse, Cell biology, ALZHEIMERS-DISEASE, medicine.anatomical_structure, HUNTINGTONS-DISEASE, Small Ubiquitin-Related Modifier Proteins, Beclin-1, Female, Signal Transduction, Programmed cell death, Patients, Mice, Transgenic, Nerve Tissue Proteins, Biology, Pathology and Forensic Medicine, ATG12, Cellular and Molecular Neuroscience, medicine, Autophagy, Animals, Humans, Spinocerebellar Ataxias, BECLIN 1, PI3K/AKT/mTOR pathway, POLYGLUTAMINE, Ataxin-7, Membrane Proteins, MUTANT ATAXIN-7, Phosphate-Binding Proteins, Gene Expression Regulation, AGGREGATE-PRONE PROTEINS, Case-Control Studies, Leukocytes, Mononuclear, Ataxia, Neurology (clinical), Transcriptome, Apoptosis Regulatory Proteins, Carrier Proteins, Lysosomes

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d0b4c82f94386243d3f8415fe0a1bc15Test
https://pubmed.ncbi.nlm.nih.gov/24859968Test

المؤلفون: Noelle Dufour, Isabel Nascimento-Ferreira, Veronica F. Colomer Gould, Tiago Santos-Ferreira, Luís Pereira de Almeida, Lígia Sousa-Ferreira, Sandro Alves, Nicole Déglon, Isabel Onofre, Arnulf H. Koeppen, Gwennaelle Auregan

المصدر: Brain : a journal of neurology. 134(Pt 5)

مصطلحات موضوعية: Male, congenital, hereditary, and neonatal diseases and abnormalities, Autophagy-Related Proteins, Mice, Transgenic, Nerve Tissue Proteins, Biology, Transfection, Mice, Sequestosome 1, Cell Line, Tumor, Sequestosome-1 Protein, medicine, Autophagy, Animals, Humans, Rats, Wistar, education, Ataxin-3, Adaptor Proteins, Signal Transducing, Aged, education.field_of_study, Neurodegeneration, Protein turnover, Brain, Membrane Proteins, Nuclear Proteins, BECN1, Machado-Joseph Disease, Middle Aged, medicine.disease, Flow Cytometry, Cell biology, Rats, Mice, Inbred C57BL, Repressor Proteins, Gene Expression Regulation, Ataxin, Immunology, Mutation, Spinocerebellar ataxia, Beclin-1, Female, Neurology (clinical), Apoptosis Regulatory Proteins, Carrier Proteins, Trinucleotide Repeat Expansion, Machado–Joseph disease

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f3dcde765af98f1eac31b546ff6a348dTest
https://pubmed.ncbi.nlm.nih.gov/21478185Test