Cargando…

MEF2 impairment underlies skeletal muscle atrophy in polyglutamine disease

Polyglutamine (polyQ) tract expansion leads to proteotoxic misfolding and drives a family of nine diseases. We study spinal and bulbar muscular atrophy (SBMA), a progressive degenerative disorder of the neuromuscular system caused by the polyQ androgen receptor (AR). Using a knock-in mouse model of...

Descripción completa

Detalles Bibliográficos
Autores principales:	Nath, Samir R., Lieberman, Matthew L., Yu, Zhigang, Marchioretti, Caterina, Jones, Samuel T., Danby, Emily C. E., Van Pelt, Kate M., Sorarù, Gianni, Robins, Diane M., Bates, Gillian P., Pennuto, Maria, Lieberman, Andrew P.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Springer Berlin Heidelberg 2020
Materias:	Original Paper
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7166004/ https://www.ncbi.nlm.nih.gov/pubmed/32306066 http://dx.doi.org/10.1007/s00401-020-02156-4

Ejemplares similares

Skeletal Muscle Pathogenesis in Polyglutamine Diseases
por: Marchioretti, Caterina, et al.
Publicado: (2022)

Introduction to the Special Issue “Skeletal Muscle Atrophy: Mechanisms at a Cellular Level”
por: Zuccaro, Emanuela, et al.
Publicado: (2023)

The Ubiquitination, Disaggregation and Proteasomal Degradation Machineries in Polyglutamine Disease
por: Nath, Samir R., et al.
Publicado: (2017)

Polyglutamine-Expanded Androgen Receptor Alteration of Skeletal Muscle Homeostasis and Myonuclear Aggregation Are Affected by Sex, Age and Muscle Metabolism
por: Chivet, Mathilde, et al.
Publicado: (2020)

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

Defective excitation-contraction coupling and mitochondrial respiration precede mitochondrial Ca(2+) accumulation in spinobulbar muscular atrophy skeletal muscle
por: Marchioretti, Caterina, et al.
Publicado: (2023)

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

Post-translational Modifications and Protein Quality Control in Motor Neuron and Polyglutamine Diseases
por: Sambataro, Fabio, et al.
Publicado: (2017)

Impaired Nuclear Export of Polyglutamine-Expanded Androgen Receptor in Spinal and Bulbar Muscular Atrophy
por: Arnold, Frederick J., et al.
Publicado: (2019)

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation
por: Wang, Adrienne M., 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)

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

Antagonistic effect of cyclin-dependent kinases and a calcium-dependent phosphatase on polyglutamine-expanded androgen receptor toxic gain of function
por: Piol, Diana, et al.
Publicado: (2023)

Microarray Analysis of Gene Expression by Skeletal Muscle of Three Mouse Models of Kennedy Disease/Spinal Bulbar Muscular Atrophy
por: Mo, Kaiguo, et al.
Publicado: (2010)

MicroRNA-142a-3p regulates neurogenic skeletal muscle atrophy by targeting Mef2a
por: Gu, Xinyi, et al.
Publicado: (2023)

LSD1/PRMT6-targeting gene therapy to attenuate androgen receptor toxic gain-of-function ameliorates spinobulbar muscular atrophy phenotypes in flies and mice
por: Prakasam, Ramachandran, et al.
Publicado: (2023)

Neuromuscular junction pathology is correlated with differential motor unit vulnerability in spinal and bulbar muscular atrophy
por: Molotsky, Elana, et al.
Publicado: (2022)

Glycolytic-to-oxidative fiber-type switch and mTOR signaling activation are early-onset features of SBMA muscle modified by high-fat diet
por: Rocchi, Anna, et al.
Publicado: (2016)

Predicted transmembrane proteins with homology to Mef(A) are not responsible for complementing mef(A) deletion in the mef(A)–msr(D) macrolide efflux system in Streptococcus pneumoniae
por: Fox, Valeria, et al.
Publicado: (2021)

Src inhibition attenuates polyglutamine-mediated neuromuscular degeneration in spinal and bulbar muscular atrophy
por: Iida, Madoka, et al.
Publicado: (2019)

The polyglutamine-expanded androgen receptor responsible for spinal and bulbar muscular atrophy inhibits the APC/C(Cdh1) ubiquitin ligase complex
por: Bott, Laura C., et al.
Publicado: (2016)

Disease Affects Bdnf Expression in Synaptic and Extrasynaptic Regions of Skeletal Muscle of Three SBMA Mouse Models
por: Halievski, Katherine, et al.
Publicado: (2019)

MEF2C and SOCS2 in stemness regulation
por: Vitali, Caterina, et al.
Publicado: (2015)

MEF2C shapes the microtranscriptome during differentiation of skeletal muscles
por: Piasecka, Agnieszka, et al.
Publicado: (2021)

Beyond motor neurons: expanding the clinical spectrum in Kennedy’s disease
por: Manzano, Raquel, et al.
Publicado: (2018)

Alopecia and cutaneous atrophy due to occipital nerve block containing steroids
por: Sadrolashrafi, Kaviyon, et al.
Publicado: (2022)

Aberrant Autophagic Response in The Muscle of A Knock-in Mouse Model of Spinal and Bulbar Muscular Atrophy
por: Rusmini, Paola, et al.
Publicado: (2015)

The role of MEF2 transcription factors in dehydration and anoxia survival in Rana sylvatica skeletal muscle
por: Hoyeck, Myriam P., et al.
Publicado: (2017)

Activin A Causes Muscle Atrophy through MEF2C-Dependent Impaired Myogenesis
por: Loumaye, Audrey, et al.
Publicado: (2022)

Autophagy and polyglutamine diseases
por: Jimenez-Sanchez, Maria, et al.
Publicado: (2012)

Polyglutamine Repeats in Viruses
por: Schein, Catherine H.
Publicado: (2018)

Mechanisms Mediating Spinal and Bulbar Muscular Atrophy: Investigations into Polyglutamine-Expanded Androgen Receptor Function and Dysfunction
por: Beitel, Lenore K., et al.
Publicado: (2013)

Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntington's Disease
por: Moffitt, Hilary, et al.
Publicado: (2009)

Macroautophagy Is Regulated by the UPR–Mediator CHOP and Accentuates the Phenotype of SBMA Mice
por: Yu, Zhigang, et al.
Publicado: (2011)

Global MEF2 target gene analysis in cardiac and skeletal muscle reveals novel regulation of DUSP6 by p38MAPK-MEF2 signaling
por: Wales, Stephanie, et al.
Publicado: (2014)

Live-cell super-resolution microscopy reveals a primary role for diffusion in polyglutamine-driven aggresome assembly
por: Lu, Meng, et al.
Publicado: (2019)

An Exploration of the Universe of Polyglutamine Structures
por: Gómez-Sicilia, Àngel, et al.
Publicado: (2015)

ANDROGEN RECEPTOR WITH SHORT POLYGLUTAMINE TRACT PREFERABLY ENHANCES WNT/β-CATENIN MEDIATED PROSTATIC TUMORIGENESIS
por: He, Yongfeng, et al.
Publicado: (2020)

Praja1 ubiquitin ligase facilitates degradation of polyglutamine proteins and suppresses polyglutamine-mediated toxicity
por: Ghosh, Baijayanti, et al.
Publicado: (2021)

AR cooperates with SMAD4 to maintain skeletal muscle homeostasis
por: Forouhan, Mitra, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_thmvao9p7kg4fmfsvjbb67rnff