| Nome |
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Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition), file dfa8b994-5f94-748b-e053-3a05fe0a3a96
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5.679
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The role of dynein mediated transport in the clearance of misfolded proteins responsible for motoneuron diseases, file dfa8b992-91ec-748b-e053-3a05fe0a3a96
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1.362
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Mutation of the SOD1 in ALS : a gain of a loss of function, file dfa8b990-bf0a-748b-e053-3a05fe0a3a96
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687
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Concurrent AFG3L2 and SPG7 mutations associated with syndromic parkinsonism and optic atrophy with aberrant OPA1 processing and mitochondrial network fragmentation, file dfa8b99b-dfba-748b-e053-3a05fe0a3a96
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561
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Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract, file dfa8b990-c389-748b-e053-3a05fe0a3a96
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559
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Trehalose induces autophagy via lysosomal-mediated TFEB activation in models of motoneuron degeneration, file dfa8b99c-3736-748b-e053-3a05fe0a3a96
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512
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The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS), file dfa8b990-80d5-748b-e053-3a05fe0a3a96
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496
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Estrogens, Neuroinflammation and Neurodegeneration, file dfa8b995-ddf6-748b-e053-3a05fe0a3a96
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435
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The anabolic/androgenic steroid nandrolone exacerbates gene expression modifications induced by mutant SOD1 in muscles of mice models of amyotrophic lateral sclerosis, file dfa8b98f-a8df-748b-e053-3a05fe0a3a96
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429
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The Role of the Heat Shock Protein B8 (HSPB8) in Motoneuron Diseases, file dfa8b998-6657-748b-e053-3a05fe0a3a96
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376
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The Androgen Derivative 5alpha-Androstan-3beta,17beta-diol Inhibits Prostate Cancer Cell Migration throught Activation of Estrogen Receptor beta subtype, file dfa8b990-91b7-748b-e053-3a05fe0a3a96
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345
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Expression of androgen-activating enzymes in cultured cells of developing rat brain, file dfa8b98f-ca3e-748b-e053-3a05fe0a3a96
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315
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Combinatorial treatment for spinal muscular atrophy, file dfa8b9a1-390f-748b-e053-3a05fe0a3a96
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302
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Transient expression of the 5alpha-reductase type 2 isozyme in the rat brain in late fetal and early postnatal life, file dfa8b98f-c707-748b-e053-3a05fe0a3a96
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259
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C9orf72 ALS/FTD dipeptide repeat protein levels are reduced by small molecules that inhibit PKA or enhance protein degradation, file dfa8b9a8-9e1e-748b-e053-3a05fe0a3a96
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251
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Role of HSPB8 in the Proteostasis Network: From Protein Synthesis to Protein Degradation and Beyond, file dfa8b993-2bc4-748b-e053-3a05fe0a3a96
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250
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The role of sex and sex Hormones in Neurodegenerative Diseases, file dfa8b99f-e982-748b-e053-3a05fe0a3a96
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250
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Inhibition of retrograde transport modulates misfolded protein accumulation and clearance in motoneuron diseases, file dfa8b998-2e38-748b-e053-3a05fe0a3a96
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236
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Androgen regulation of axon growth and neurite extension in motoneurons, file dfa8b98f-af5b-748b-e053-3a05fe0a3a96
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234
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Differential autophagy power in the spinal cord and muscle of transgenic ALS mice, file dfa8b990-dfde-748b-e053-3a05fe0a3a96
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229
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The regulation of the small heat shock protein B8 in misfolding protein diseases causing motoneuronal and muscle cell death, file dfa8b99f-2f92-748b-e053-3a05fe0a3a96
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189
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The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases, file dfa8b998-8731-748b-e053-3a05fe0a3a96
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185
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Dual role of autophagy on docetaxel-sensitivity in prostate cancer cells, file dfa8b99b-b917-748b-e053-3a05fe0a3a96
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184
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FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy, file dfa8b99d-fd94-748b-e053-3a05fe0a3a96
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180
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Human adipose-derived mesenchymal stem cells as a new model of spinal and bulbar muscular atrophy, file dfa8b991-694d-748b-e053-3a05fe0a3a96
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178
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The chaperone-assisted selective autophagy complex dynamics and dysfunctions, file d9a1633e-95f7-4486-b7a9-2993a0f19cef
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174
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Post-translational modifications and expanded polyglutamine toxicity in neurons, file dfa8b990-c6d6-748b-e053-3a05fe0a3a96
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174
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Nuclear Phospho-SOD1 Protects DNA from Oxidative Stress Damage in Amyotrophic Lateral Sclerosis, file dfa8b99e-4e13-748b-e053-3a05fe0a3a96
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172
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Insights into the neurodegenerative mechanisms associated with KIF5A mutations, file 56680d93-303f-47df-b795-36bf6c8530d4
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164
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Transcriptional induction of the heat shock protein B8 mediates the clearance of misfolded proteins responsible for motor neuron diseases, file dfa8b995-01cf-748b-e053-3a05fe0a3a96
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164
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Proteostasis and ALS : protocol for a phase II, randomised, double-blind, placebo-controlled, multicentre clinical trial for colchicine in ALS (Co-ALS), file dfa8b99e-5376-748b-e053-3a05fe0a3a96
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162
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Pathological Proteins Are Transported by Extracellular Vesicles of Sporadic Amyotrophic Lateral Sclerosis Patients, file dfa8b99c-07e8-748b-e053-3a05fe0a3a96
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156
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Aberrant Autophagic Response in The Muscle of A Knock-in Mouse Model of Spinal and Bulbar Muscular Atrophy, file dfa8b993-1c77-748b-e053-3a05fe0a3a96
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154
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Muscle Cells : Motor Neuron Targets and Contributors of their Disorders, file dfa8b994-7b34-748b-e053-3a05fe0a3a96
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151
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Dysfunction of constitutive and inducible ubiquitin-proteasome system in amyotrophic lateral sclerosis: Implication for protein aggregation and immune response, file dfa8b990-5652-748b-e053-3a05fe0a3a96
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147
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The small heat shock protein B8 (HSPB8) modulates proliferation and migration of breast cancer cells, file dfa8b997-422e-748b-e053-3a05fe0a3a96
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147
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How PQC inhibition modulates miRNA loading in large and small extracellular vesicles, file 21b0955f-899c-471d-9dd4-58f91281cc38
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144
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Tdp-25 Routing to Autophagy and Proteasome Ameliorates its Aggregation in Amyotrophic Lateral Sclerosis Target Cells, file dfa8b99b-b6d6-748b-e053-3a05fe0a3a96
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144
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The Role of HSPB8, a Component of the Chaperone-Assisted Selective Autophagy Machinery, in Cancer, file dfa8b9a5-4190-748b-e053-3a05fe0a3a96
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144
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Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice, file dfa8b999-5071-748b-e053-3a05fe0a3a96
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141
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The protein quality control system in motoneuron diseases, file dfa8b992-881d-748b-e053-3a05fe0a3a96
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136
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Autophagic and Proteasomal Mediated Removal of Mutant Androgen Receptor in Muscle Models of Spinal and Bulbar Muscular Atrophy, file dfa8b99f-4adc-748b-e053-3a05fe0a3a96
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134
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Autophagy in neurodegeneration: New insights underpinning therapy for neurological diseases, file dfa8b9a1-7531-748b-e053-3a05fe0a3a96
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116
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Dysregulation of Muscle-Specific MicroRNAs as Common Pathogenic Feature Associated with Muscle Atrophy in ALS, SMA and SBMA: Evidence from Animal Models and Human Patients, file dfa8b9a6-f3cf-748b-e053-3a05fe0a3a96
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116
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Alteration of the protein quality control system in motor neuron and muscle expressing mutant proteins causing ALS and SBMA, file dfa8b992-9759-748b-e053-3a05fe0a3a96
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115
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BAG3 Pro209 mutants associated with myopathy and neuropathy relocate chaperones of the CASA-complex to aggresomes, file dfa8b9a2-5e47-748b-e053-3a05fe0a3a96
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114
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Transforming Growth Factor beta 1 signaling is altered in the spinal cord and muscle of amyotrophic lateral sclerosis mice and patients, file dfa8b99e-ae71-748b-e053-3a05fe0a3a96
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113
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Enhanced Clearance of Neurotoxic Misfolded Proteins by the Natural Compound Berberine and Its Derivatives, file dfa8b9a1-f092-748b-e053-3a05fe0a3a96
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107
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A Crucial Role for the Protein Quality Control System in Motor Neuron Diseases, file dfa8b9a3-0fed-748b-e053-3a05fe0a3a96
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107
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Multiple Roles of Transforming Growth Factor Beta in Amyotrophic Lateral Sclerosis, file dfa8b9a2-301d-748b-e053-3a05fe0a3a96
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105
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Retinoic Acid Downregulates HSPB8 Gene Expression in Human Breast Cancer Cells MCF-7, file dfa8b9a6-e4c0-748b-e053-3a05fe0a3a96
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104
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Neurodegenerative Disease-Associated TDP-43 Fragments Are Extracellularly Secreted with CASA Complex Proteins, file dfa8b9a9-da13-748b-e053-3a05fe0a3a96
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101
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Pathogenic variants of Valosin Containing Protein induce lysosomal damage and transcriptional activation of autophagy regulators in neuronal cells, file e9d18bb1-67a8-43d4-aa12-75d15b29ae6f
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97
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Loss-of-function mutations in the SIGMAR1 gene cause distal hereditary motor neuropathy by impairing ER-mitochondria tethering and Ca2+ signaling, file dfa8b9a6-d8ff-748b-e053-3a05fe0a3a96
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95
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TDP-43 PROTEINOPATHIES: THE ROLE OF EXTRACELLULAR VESICLES, file 27dc34a9-3d27-48c5-a369-e2d3c832a3ec
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92
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Trehalose induces autophagy via lysosomal-mediated TFEB activation in models of motoneuron degeneration, file dfa8b99d-29d3-748b-e053-3a05fe0a3a96
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87
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Differential autophagy power in the spinal cord and muscle of transgenic ALS mice, file dfa8b991-3d05-748b-e053-3a05fe0a3a96
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82
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A Surveillance Function of the HSPB8-BAG3-HSP70 Chaperone complex ensures stress granule integrity and dynamism, file dfa8b995-ea60-748b-e053-3a05fe0a3a96
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82
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Multilayer and MATR3-dependent regulation of mRNAs maintains pluripotency in human induced pluripotent stem cells, file dfa8b9a7-310d-748b-e053-3a05fe0a3a96
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82
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Insights into KIF5A-related pathways to neurodegeneration, file 138261e4-6e69-4f83-a528-4b5dd6c3c8d9
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77
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One gene, many phenotypes: investigating KIF5A-linked neurodegeneration mechanisms, file 86e3e1a7-695b-431f-8637-378366a4b8c6
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70
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Insights on human small heat shock proteins and their alterations in diseases, file dfa8b9a9-ad26-748b-e053-3a05fe0a3a96
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67
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Isogenic FUS-eGFP iPSC Reporter Lines Enable Quantification of FUS Stress Granule Pathology that Is Rescued by Drugs Inducing Autophagy, file dfa8b99a-e39f-748b-e053-3a05fe0a3a96
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63
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Synergic prodegradative activity of bicalutamide and trehalose on the mutant androgen receptor responsible for spinal and bulbar muscular atrophy, file dfa8b995-32a4-748b-e053-3a05fe0a3a96
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61
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The physiopathological roles of androgens in motoneurons, file dfa8b9a1-582c-748b-e053-3a05fe0a3a96
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59
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Effects and metabolism of steroid hormones in human neuroblastoma cells, file dfa8b98f-ebd8-748b-e053-3a05fe0a3a96
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56
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The role of autophagy-lysosomal pathway in motor neuron diseases, file bbf9e809-278b-41af-9e60-753362497db9
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51
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BAG1 prevents misfolded proteins accumulation when autophagy flux is blocked in neurodegenerative disorders, file dfa8b999-5868-748b-e053-3a05fe0a3a96
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51
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Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy, file dfa8b9a9-a227-748b-e053-3a05fe0a3a96
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51
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RNA molecular signature profiling in PBMCs of sporadic ALS patients: HSP70 overexpression is associated with nuclear SOD1, file dfa8b9a9-6e2e-748b-e053-3a05fe0a3a96
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50
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HSPB8 frameshift mutant aggregates weaken chaperone-assisted selective autophagy in neuromyopathies, file 4643b012-2d73-4531-9774-06eb91ee2634
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49
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Identification of HSPB8 modulators counteracting misfolded protein accumulation in neurodegenerative diseases, file be05b08b-f354-4c6b-8379-4823c368429d
|
47
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|
TDP25 aggregation in motor neuron and muscle cells is rescued by chaperone overexpression, file dfa8b99c-60cc-748b-e053-3a05fe0a3a96
|
46
|
|
Large and small extracellular vesicles may contribute to the propagation of ALS and FTD carrying toxic TDP species and potentially harmful miRNAs, file 35c1fa66-e63a-4b28-9e47-290afca853c5
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45
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5 alpha-reductase isozymes in the central nervous system, file dfa8b98f-ce85-748b-e053-3a05fe0a3a96
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44
|
|
Autophagy in neurodegeneration: New insights underpinning therapy for neurological diseases, file dfa8b9a2-0eb2-748b-e053-3a05fe0a3a96
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44
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|
Bicalutamide and Trehalose Ameliorate Spinal and Bulbar Muscular Atrophy Pathology in Mice, file d3a6c3cf-972a-4e04-b1d2-a62aba6a58be
|
43
|
|
Amyotrophic lateral sclerosis related proteins are actively removed by HspB8, file dfa8b995-33d9-748b-e053-3a05fe0a3a96
|
42
|
|
Androgen metabolism in different target tissues, file dfa8b98f-df07-748b-e053-3a05fe0a3a96
|
35
|
|
Identification of type 1 5alpha-reductase in myelin membranes of male and female rat brain, file dfa8b98f-d734-748b-e053-3a05fe0a3a96
|
34
|
|
Role of the autophagic pathway in a muscle model of Spinal and Bulbar Muscular Atrophy, file dfa8b995-09d8-748b-e053-3a05fe0a3a96
|
34
|
|
Analysis of the pathogenetic mechanisms involved in KIF5A-related neurodegenerative disorders, file dfa8b9a9-f163-748b-e053-3a05fe0a3a96
|
34
|
|
The role of extracellular vesicles in TDP-43 proteinopathies, file 8550b912-bba9-4b20-a47c-685326e8edf0
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31
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The role of extracellular vesicles in the removal of aggregated TDP43 responsible for ALS/FTD diseases, file dfa8b9a0-31b9-748b-e053-3a05fe0a3a96
|
31
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|
Mechanism of paroxetine-mediated autophagic induction in cell models of ALS/FTD, file 680b2e1a-fd35-472a-a8e0-6f6d4a0a8f06
|
30
|
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RAN translated C9ORF72 arginine rich poly-dipeptides alter gene transcription in ALS/FTD cell model, file dfa8b9a0-202c-748b-e053-3a05fe0a3a96
|
29
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|
Shared behaviours of KIF5A frameshift mutants in neurodevelopment and neurodegeneration, file d000fe03-ec7f-423f-9234-72bc7c9c72a9
|
28
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|
The contribution of protein quality control in the pathogenesis of SBMA, file dfa8b992-80fa-748b-e053-3a05fe0a3a96
|
28
|
|
The role of the PQC system in managing misfolded protein in motoneuronal and muscular models of ALS and SBMA, file dfa8b992-be5b-748b-e053-3a05fe0a3a96
|
28
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The Role of Small Heat Shock Proteins in Protein Misfolding Associated Motoneuron Diseases, file bf74631b-d95e-48ae-995b-2f70e5976179
|
25
|
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Ontogenetic development of the 5 alpha-reductase in the rat brain: cerebral cortex, hypothalamus, purified myelin and isolated oligodendrocytes, file dfa8b98f-d7ac-748b-e053-3a05fe0a3a96
|
25
|
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TDP43 inclusions are re-routes to autophagy by the activity of the small chaperone HspB8, file dfa8b995-82d1-748b-e053-3a05fe0a3a96
|
25
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VCP mutants cause lysosomal alterations and autophagy induction in ALS-neuronal model, file dfa8b9a7-aec2-748b-e053-3a05fe0a3a96
|
24
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Geldanamycin and spironolactone enhance the degradationofC9orf72ALS/FTDdipeptiderepeatproteins, file 100a3bb7-34dd-42a4-bc95-b0cf900c8cee
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23
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Inhibition of retrograde transport modulates misfolded protein accumulation and clearance in motoneuron diseases, file dfa8b9a6-bb65-748b-e053-3a05fe0a3a96
|
23
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Deepening the characterization and behaviour of mutants of the Bcl-2 Associated Athanogene 3 (BAG3), file dfa8b9a0-d79c-748b-e053-3a05fe0a3a96
|
22
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Novel pathogenic mechanism of ALS-associated VCP- mutants, file dfa8b99e-4c66-748b-e053-3a05fe0a3a96
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21
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VCP: a novel regulator of SOD1-G93A clearance in a als model, file dfa8b99a-daef-748b-e053-3a05fe0a3a96
|
20
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Mutations in VCP induce lysosomal alterations and autophagy activation in ALS neuronal models, file dfa8b9a7-6e91-748b-e053-3a05fe0a3a96
|
20
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Motor neuron degeneration in spinal and bulbar muscular atrophy: molecular approaches to counteract mutant androgen receptor neurotoxicity, file dfa8b9a0-408e-748b-e053-3a05fe0a3a96
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19
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| Totale |
20.611 |