C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal

C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal

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Genetic Correction of SOD1 Mutant iPSCs Reveals ERK and JNK Activated AP1 as a Driver of Neurodegeneration in Amyotrophic Lateral Sclerosis.Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration Amyotrophic lateral sclerosis patient iPSC-derived astrocytes impair autophagy via non-cell autonomous mechanisms.Abnormalities of Mitochondrial Dynamics in Neurodegenerative Diseases.Variant U1 snRNAs are implicated in human pluripotent stem cell maintenance and neuromuscular disease.C9orf72 and RAB7L1 regulate vesicle trafficking in amyotrophic lateral sclerosis and frontotemporal dementia.ER stress and the unfolded protein response in neurodegeneration.Modeling the C9ORF72 repeat expansion mutation using human induced pluripotent stem cells.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.Energy Homeostasis and Abnormal RNA Metabolism in Amyotrophic Lateral Sclerosis The Endoplasmic Reticulum Unfolded Protein Response in Neurodegenerative Disorders and Its Potential Therapeutic Significance Multifunctional molecule ERp57: From cancer to neurodegenerative diseases.C9ORF72 is a GDP/GTP exchange factor for Rab8 and Rab39 and regulates autophagy.Fine-Tuning ER Stress Signal Transducers to Treat Amyotrophic Lateral Sclerosis.The Role of BiP Retrieval by the KDEL Receptor in the Early Secretory Pathway and its Effect on Protein Quality Control and Neurodegeneration.MAPT Genetic Variation and Neuronal Maturity Alter Isoform Expression Affecting Axonal Transport in iPSC-Derived Dopamine Neurons.Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS.Excess α-synuclein compromises phagocytosis in iPSC-derived macrophages Proteostasis disturbance in amyotrophic lateral sclerosis.Neuroprotective Potential of Cell-Based Therapies in ALS: From Bench to Bedside.A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism.RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration.Calcium dysregulation and Cdk5-ATM pathway involved in a mouse model of fragile X-associated tremor/ataxia syndrome.C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity.Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells.C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylation.A Simplified Method for Generating Purkinje Cells from Human-Induced Pluripotent Stem Cells.Alzheimer's Disease: A Special Collection.Synaptic dysfunction and altered excitability in C9ORF72 ALS/FTD.Stem Cell modeling of Mitochondrial Parkinsonism reveals key functions of OPA1.CRISPR-Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity.2D versus 3D human induced pluripotent stem cell-derived cultures for neurodegenerative disease modelling.Modeling Protein Aggregation and the Heat Shock Response in ALS iPSC-Derived Motor Neurons.Neurodegeneration in SCA14 is associated with increased PKCγ kinase activity, mislocalization and aggregation RIPK1 is a critical modulator of both tonic and TLR-responsive inflammatory and cell death pathways in human macrophage differentiation

P2860

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P2860

C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal

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scientific article published on 20 April 2016

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