Cargando…

Aberrant Autophagic Response in The Muscle of A Knock-in Mouse Model of Spinal and Bulbar Muscular Atrophy

Spinal and bulbar muscular atrophy (SBMA) is characterized by loss of motoneurons and sensory neurons, accompanied by atrophy of muscle cells. SBMA is due to an androgen receptor containing a polyglutamine tract (ARpolyQ) that misfolds and aggregates, thereby perturbing the protein quality control (...

Descripción completa

Detalles Bibliográficos
Autores principales:	Rusmini, Paola, Polanco, Maria Josefa, Cristofani, Riccardo, Cicardi, Maria Elena, Meroni, Marco, Galbiati, Mariarita, Piccolella, Margherita, Messi, Elio, Giorgetti, Elisa, Lieberman, Andrew P., Milioto, Carmelo, Rocchi, Anna, Aggarwal, Tanya, Pennuto, Maria, Crippa, Valeria, Poletti, Angelo
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group 2015
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4614888/ https://www.ncbi.nlm.nih.gov/pubmed/26490709 http://dx.doi.org/10.1038/srep15174

Ejemplares similares

Autophagic and Proteasomal Mediated Removal of Mutant Androgen Receptor in Muscle Models of Spinal and Bulbar Muscular Atrophy
por: Cicardi, Maria Elena, et al.
Publicado: (2019)

Synergic prodegradative activity of Bicalutamide and trehalose on the mutant androgen receptor responsible for spinal and bulbar muscular atrophy
por: Giorgetti, Elisa, et al.
Publicado: (2015)

The protein quality control system in motoneuron diseases
por: Poletti, Angelo, et al.
Publicado: (2015)

The small heat shock protein B8 (HSPB8) modulates proliferation and migration of breast cancer cells
por: Piccolella, Margherita, et al.
Publicado: (2017)

The role of dynein mediated transport in the clearance of misfolded proteins responsible for motoneuron diseases
por: Cristofani, Riccardo, et al.
Publicado: (2015)

The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases
por: Cristofani, Riccardo, et al.
Publicado: (2017)

The Role of the Heat Shock Protein B8 (HSPB8) in Motoneuron Diseases
por: Rusmini, Paola, et al.
Publicado: (2017)

Inhibition of retrograde transport modulates misfolded protein accumulation and clearance in motoneuron diseases
por: Cristofani, Riccardo, et al.
Publicado: (2017)

A Crucial Role for the Protein Quality Control System in Motor Neuron Diseases
por: Cristofani, Riccardo, et al.
Publicado: (2020)

Bicalutamide and Trehalose Ameliorate Spinal and Bulbar Muscular Atrophy Pathology in Mice
por: Galbiati, Mariarita, et al.
Publicado: (2023)

Tdp-25 Routing to Autophagy and Proteasome Ameliorates its Aggregation in Amyotrophic Lateral Sclerosis Target Cells
por: Cicardi, Maria Elena, et al.
Publicado: (2018)

The Regulation of the Small Heat Shock Protein B8 in Misfolding Protein Diseases Causing Motoneuronal and Muscle Cell Death
por: Cristofani, Riccardo, et al.
Publicado: (2019)

Enhanced Clearance of Neurotoxic Misfolded Proteins by the Natural Compound Berberine and Its Derivatives
por: Rusmini, Paola, et al.
Publicado: (2020)

Differential autophagy power in the spinal cord and muscle of transgenic ALS mice
por: Crippa, Valeria, et al.
Publicado: (2013)

Transcriptional induction of the heat shock protein B8 mediates the clearance of misfolded proteins responsible for motor neuron diseases
por: Crippa, Valeria, et al.
Publicado: (2016)

Multiple Roles of Transforming Growth Factor Beta in Amyotrophic Lateral Sclerosis
por: Galbiati, Mariarita, et al.
Publicado: (2020)

Clearance of the mutant androgen receptor in motoneuronal models of spinal and bulbar muscular atrophy()
por: Rusmini, Paola, et al.
Publicado: (2013)

Retinoic Acid Downregulates HSPB8 Gene Expression in Human Breast Cancer Cells MCF-7
por: Piccolella, Margherita, et al.
Publicado: (2021)

Alteration of the protein quality control system in motor neuron and muscle expressing mutant proteins causing ALS and SBMA
por: Cicardi, Maria Elena, et al.
Publicado: (2015)

Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy
por: Ferrari, Veronica, et al.
Publicado: (2022)

The role of autophagy-lysosomal pathway in motor neuron diseases
por: Tedesco, Barbara, et al.
Publicado: (2022)

Beta-agonist stimulation ameliorates the phenotype of spinal and bulbar muscular atrophy mice and patient-derived myotubes
por: Milioto, Carmelo, et al.
Publicado: (2017)

Insights on Human Small Heat Shock Proteins and Their Alterations in Diseases
por: Tedesco, B., et al.
Publicado: (2022)

Pathogenic variants of Valosin-containing protein induce lysosomal damage and transcriptional activation of autophagy regulators in neuronal cells
por: Ferrari, Veronica, et al.
Publicado: (2022)

Muscle cells and motoneurons differentially remove mutant SOD1 causing familial amyotrophic lateral sclerosis
por: Onesto, Elisa, et al.
Publicado: (2011)

The Role of Small Heat Shock Proteins in Protein Misfolding Associated Motoneuron Diseases
por: Tedesco, Barbara, et al.
Publicado: (2022)

Neurodegenerative Disease-Associated TDP-43 Fragments Are Extracellularly Secreted with CASA Complex Proteins
por: Casarotto, Elena, et al.
Publicado: (2022)

The anabolic/androgenic steroid nandrolone exacerbates gene expression modifications induced by mutant SOD1 in muscles of mice models of amyotrophic lateral sclerosis
por: Galbiati, Mariarita, et al.
Publicado: (2012)

Protein Arginine Methyltransferase 6 Enhances Polyglutamine-Expanded Androgen Receptor Function and Toxicity in Spinal and Bulbar Muscular Atrophy
por: Scaramuzzino, Chiara, et al.
Publicado: (2015)

Mitochondrial abnormalities in spinal and bulbar muscular atrophy
por: Ranganathan, Srikanth, et al.
Publicado: (2009)

Editorial Comment to Castration‐resistant prostate cancer diagnosed during leuprorelin treatment for spinal and bulbar muscular atrophy
por: Pennuto, Maria, et al.
Publicado: (2022)

The Role of HSPB8, a Component of the Chaperone-Assisted Selective Autophagy Machinery, in Cancer
por: Cristofani, Riccardo, et al.
Publicado: (2021)

Human Adipose-Derived Mesenchymal Stem Cells as a New Model of Spinal and Bulbar Muscular Atrophy
por: Dossena, Marta, et al.
Publicado: (2014)

Increased mitophagy in the skeletal muscle of spinal and bulbar muscular atrophy patients
por: Borgia, Doriana, et al.
Publicado: (2017)

HSPB8 frameshift mutant aggregates weaken chaperone-assisted selective autophagy in neuromyopathies
por: Tedesco, Barbara, et al.
Publicado: (2023)

The value of serum creatinine as biomarker of disease progression in spinal and bulbar muscular atrophy (SBMA)
por: Blasi, Lorenzo, et al.
Publicado: (2023)

HSPB8 counteracts tumor activity of BRAF- and NRAS-mutant melanoma cells by modulation of RAS-prenylation and autophagy
por: Cristofani, Riccardo, et al.
Publicado: (2022)

Dual role of autophagy on docetaxel-sensitivity in prostate cancer cells
por: Cristofani, Riccardo, et al.
Publicado: (2018)

Bulbar Paralysis
Publicado: (1876)

Non-neural phenotype of spinal and bulbar muscular atrophy: results from a large cohort of Italian patients
por: Querin, Giorgia, et al.
Publicado: (2016)

Cannot write session to /tmp/vufind_sessions/sess_hse6f4k0ded928plfh55s50bog