Cargando…

Mitochondrial Dysregulation and Impaired Autophagy in iPSC-Derived Dopaminergic Neurons of Multiple System Atrophy

Multiple system atrophy (MSA) is a progressive neurodegenerative disease that affects several areas of the CNS, whose pathogenesis is still widely unclear and for which an effective treatment is lacking. We have generated induced pluripotent stem cell-derived dopaminergic neurons from four MSA patie...

Descripción completa

Detalles Bibliográficos
Autores principales:	Monzio Compagnoni, Giacomo, Kleiner, Giulio, Samarani, Maura, Aureli, Massimo, Faustini, Gaia, Bellucci, Arianna, Ronchi, Dario, Bordoni, Andreina, Garbellini, Manuela, Salani, Sabrina, Fortunato, Francesco, Frattini, Emanuele, Abati, Elena, Bergamini, Christian, Fato, Romana, Tabano, Silvia, Miozzo, Monica, Serratto, Giulia, Passafaro, Maria, Deleidi, Michela, Silipigni, Rosamaria, Nizzardo, Monica, Bresolin, Nereo, Comi, Giacomo P., Corti, Stefania, Quinzii, Catarina M., Di Fonzo, Alessio
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Elsevier 2018
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234905/ https://www.ncbi.nlm.nih.gov/pubmed/30344007 http://dx.doi.org/10.1016/j.stemcr.2018.09.007

Ejemplares similares

Understanding the pathogenesis of multiple system atrophy: state of the art and future perspectives
por: Monzio Compagnoni, Giacomo, et al.
Publicado: (2019)

Spinal muscular atrophy phenotype is ameliorated in human motor neurons by SMN increase via different novel RNA therapeutic approaches
por: Nizzardo, Monica, et al.
Publicado: (2015)

Animal Models of CMT2A: State-of-art and Therapeutic Implications
por: De Gioia, Roberta, et al.
Publicado: (2020)

iPSC-Derived Neural Stem Cells Act via Kinase Inhibition to Exert Neuroprotective Effects in Spinal Muscular Atrophy with Respiratory Distress Type 1
por: Simone, Chiara, et al.
Publicado: (2014)

Cell-penetrating peptide-conjugated Morpholino rescues SMA in a symptomatic preclinical model
por: Bersani, Margherita, et al.
Publicado: (2022)

Neural Stem Cell Transplantation for Neurodegenerative Diseases
por: De Gioia, Roberta, et al.
Publicado: (2020)

A Novel Homozygous VPS11 Variant May Cause Generalized Dystonia
por: Monfrini, Edoardo, et al.
Publicado: (2021)

Late-onset leukoencephalopathy in a patient with recessive EARS2 mutations
por: Monfrini, Edoardo, et al.
Publicado: (2020)

Minimally invasive transplantation of iPSC-derived ALDHhiSSCloVLA4+ neural stem cells effectively improves the phenotype of an amyotrophic lateral sclerosis model
por: Nizzardo, Monica, et al.
Publicado: (2014)

Combined RNA interference and gene replacement therapy targeting MFN2 as proof of principle for the treatment of Charcot–Marie–Tooth type 2A
por: Rizzo, Federica, et al.
Publicado: (2023)

Spinal muscular atrophy with respiratory distress type 1: Clinical phenotypes, molecular pathogenesis and therapeutic insights
por: Saladini, Matteo, et al.
Publicado: (2019)

Investigation of New Morpholino Oligomers to Increase Survival Motor Neuron Protein Levels in Spinal Muscular Atrophy
por: Ramirez, Agnese, et al.
Publicado: (2018)

Gene therapy rescues disease phenotype in a spinal muscular atrophy with respiratory distress type 1 (SMARD1) mouse model
por: Nizzardo, Monica, et al.
Publicado: (2015)

Genome-wide RNA-seq of iPSC-derived motor neurons indicates selective cytoskeletal perturbation in Brown–Vialetto disease that is partially rescued by riboflavin
por: Rizzo, Federica, et al.
Publicado: (2017)

Molecular, genetic and stem cell-mediated therapeutic strategies for spinal muscular atrophy (SMA)
por: Zanetta, Chiara, et al.
Publicado: (2014)

Management of patients with neuromuscular disorders at the time of the SARS-CoV-2 pandemic
por: Costamagna, Gianluca, et al.
Publicado: (2020)

Direct reprogramming of human astrocytes into neural stem cells and neurons
por: Corti, Stefania, et al.
Publicado: (2012)

Dystonia‐ataxia syndrome with permanent torsional nystagmus caused by ECHS1 deficiency
por: Ronchi, Dario, et al.
Publicado: (2020)

Key role of SMN/SYNCRIP and RNA-Motif 7 in spinal muscular atrophy: RNA-Seq and motif analysis of human motor neurons
por: Rizzo, Federica, et al.
Publicado: (2019)

Generation of skeletal muscle cells from embryonic and induced pluripotent stem cells as an in vitro model and for therapy of muscular dystrophies
por: Salani, Sabrina, et al.
Publicado: (2012)

Antisense Morpholino-Based In Vitro Correction of a Pseudoexon-Generating Variant in the SGCB Gene
por: Magri, Francesca, et al.
Publicado: (2022)

Transcriptomic characterization of tissues from patients and subsequent pathway analyses reveal biological pathways that are implicated in spastic ataxia
por: Kakouri, Andrea C., et al.
Publicado: (2022)

The GBAP1 pseudogene acts as a ceRNA for the glucocerebrosidase gene GBA by sponging miR-22-3p
por: Straniero, Letizia, et al.
Publicado: (2017)

MicroRNA expression analysis identifies a subset of downregulated miRNAs in ALS motor neuron progenitors
por: Rizzuti, Mafalda, et al.
Publicado: (2018)

Insights into the identification of a molecular signature for amyotrophic lateral sclerosis exploiting integrated microRNA profiling of iPSC-derived motor neurons and exosomes
por: Rizzuti, Mafalda, et al.
Publicado: (2022)

Human induced pluripotent stem cell models for the study and treatment of Duchenne and Becker muscular dystrophies
por: Piga, Daniela, et al.
Publicado: (2019)

Can Intestinal Pseudo-Obstruction Drive Recurrent Stroke-Like Episodes in Late-Onset MELAS Syndrome? A Case Report and Review of the Literature
por: Gagliardi, Delia, et al.
Publicado: (2019)

Beckwith–Wiedemann and IMAGe syndromes: two very different diseases caused by mutations on the same gene
por: Milani, Donatella, et al.
Publicado: (2014)

Charcot–Marie–Tooth disease type 2F associated with biallelic HSPB1 mutations
por: Abati, Elena, et al.
Publicado: (2021)

Two novel mutations in PEO1 (Twinkle) gene associated with chronic external ophthalmoplegia()
por: Ronchi, Dario, et al.
Publicado: (2011)

Beckwith–Wiedemann syndrome prenatal diagnosis by methylation analysis in chorionic villi
por: Paganini, Leda, et al.
Publicado: (2015)

Mitochondrial Fusion Proteins and Human Diseases
por: Ranieri, Michela, et al.
Publicado: (2013)

A novel CCM1 mutation associated with multiple cerebral and vertebral cavernous malformations
por: Lanfranconi, Silvia, et al.
Publicado: (2014)

A case report of late-onset cerebellar ataxia associated with a rare p.R342W TGM6 (SCA35) mutation
por: Manini, Arianna, et al.
Publicado: (2020)

MicroRNAs as regulators of cell death mechanisms in amyotrophic lateral sclerosis
por: Gagliardi, Delia, et al.
Publicado: (2019)

Extracellular vesicles and amyotrophic lateral sclerosis: from misfolded protein vehicles to promising clinical biomarkers
por: Gagliardi, Delia, et al.
Publicado: (2020)

Hyperacute extensive spinal cord infarction and negative spine magnetic resonance imaging: a case report and review of the literature
por: Costamagna, Gianluca, et al.
Publicado: (2020)

Mass spectrometry-based assay for the molecular diagnosis of glioma: concomitant detection of chromosome 1p/19q codeletion, and IDH1, IDH2, and TERT mutation status
por: Pesenti, Chiara, et al.
Publicado: (2017)

Fragile X syndrome: a review of clinical and molecular diagnoses
por: Ciaccio, Claudia, et al.
Publicado: (2017)

Ophthalmoplegia Due to Miller Fisher Syndrome in a Patient With Myasthenia Gravis
por: Brusa, Roberta, et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_i1kltmu2v920g8thf3i2fg7big