Cargando…

Spinal Muscular Atrophy autophagy profile is tissue-dependent: differential regulation between muscle and motoneurons

Spinal muscular atrophy (SMA) is a neuromuscular genetic disease caused by reduced survival motor neuron (SMN) protein. SMN is ubiquitous and deficient levels cause spinal cord motoneurons (MNs) degeneration and muscle atrophy. Nevertheless, the mechanism by which SMN reduction in muscle contributes...

Descripción completa

Detalles Bibliográficos
Autores principales:	Sansa, Alba, Hidalgo, Ivan, Miralles, Maria P., de la Fuente, Sandra, Perez-Garcia, M. Jose, Munell, Francina, Soler, Rosa M., Garcera, Ana
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	BioMed Central 2021
Materias:	Research
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8254901/ https://www.ncbi.nlm.nih.gov/pubmed/34217376 http://dx.doi.org/10.1186/s40478-021-01223-5

Ejemplares similares

Calpain Inhibition Increases SMN Protein in Spinal Cord Motoneurons and Ameliorates the Spinal Muscular Atrophy Phenotype in Mice
por: de la Fuente, Sandra, et al.
Publicado: (2018)

Survival motor neuron protein and neurite degeneration are regulated by Gemin3 in spinal muscular atrophy motoneurons
por: Miralles, Maria P., et al.
Publicado: (2022)

ERK MAPK signaling pathway inhibition as a potential target to prevent autophagy alterations in Spinal Muscular Atrophy motoneurons
por: Sansa, Alba, et al.
Publicado: (2023)

Notch Signaling Pathway Is Activated in Motoneurons of Spinal Muscular Atrophy
por: Caraballo-Miralles, Víctor, et al.
Publicado: (2013)

Calpain system is altered in survival motor neuron-reduced cells from in vitro and in vivo spinal muscular atrophy models
por: de la Fuente, Sandra, et al.
Publicado: (2020)

Inhibition of autophagy delays motoneuron degeneration and extends lifespan in a mouse model of spinal muscular atrophy
por: Piras, Antonio, et al.
Publicado: (2017)

Survival motor neuron protein reduction deregulates autophagy in spinal cord motoneurons in vitro
por: Garcera, A, et al.
Publicado: (2013)

Smn-Deficiency Increases the Intrinsic Excitability of Motoneurons
por: Arumugam, Saravanan, et al.
Publicado: (2017)

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

R-Roscovitine Improves Motoneuron Function in Mouse Models for Spinal Muscular Atrophy
por: Tejero, Rocio, et al.
Publicado: (2020)

Defective Ca(2+) channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophy
por: Jablonka, Sibylle, et al.
Publicado: (2007)

Spinal muscular atrophy
por: D'Amico, Adele, et al.
Publicado: (2011)

Respiratory muscle fatigability in patients with spinal muscular atrophy
por: Kant‐Smits, Kim, et al.
Publicado: (2022)

Diminished muscle oxygen uptake and fatigue in spinal muscular atrophy
por: Montes, Jacqueline, et al.
Publicado: (2021)

Autophagy inhibition: a new therapeutic target in spinal muscular atrophy
por: Piras, Antonio, et al.
Publicado: (2018)

Neuronal activity regulates DROSHA via autophagy in spinal muscular atrophy
por: Gonçalves, Inês do Carmo G., et al.
Publicado: (2018)

Impaired muscle uptake of creatine in spinal and bulbar muscular atrophy
por: Hijikata, Yasuhiro, et al.
Publicado: (2016)

Nusinersen for spinal muscular atrophy
por: Wurster, Claudia D., et al.
Publicado: (2018)

Spinal Muscular Atrophy and Ependymoma
por: Albakr, Aishah, et al.
Publicado: (2017)

Risdiplam for spinal muscular atrophy
Publicado: (2022)

Differential induction of muscle atrophy pathways in two mouse models of spinal muscular atrophy
por: Deguise, Marc-Olivier, et al.
Publicado: (2016)

Increasing Agrin Function Antagonizes Muscle Atrophy and Motor Impairment in Spinal Muscular Atrophy
por: Boido, Marina, et al.
Publicado: (2018)

Motoneuronal Spinal Circuits in Degenerative Motoneuron Disease
por: Falgairolle, Mélanie, et al.
Publicado: (2020)

Magnetic resonance reveals mitochondrial dysfunction and muscle remodelling in spinal muscular atrophy
por: Habets, Laura E, et al.
Publicado: (2021)

Smn, the spinal muscular atrophy–determining gene product, modulates axon growth and localization of β-actin mRNA in growth cones of motoneurons
por: Rossoll, Wilfried, et al.
Publicado: (2003)

Early electrical field stimulation prevents the loss of spinal cord anterior horn motoneurons and muscle atrophy following spinal cord injury
por: Zhang, Cheng, et al.
Publicado: (2018)

Different atrophy-hypertrophy transcription pathways in muscles affected by severe and mild spinal muscular atrophy
por: Millino, Caterina, et al.
Publicado: (2009)

Muscle and not neuronal biomarkers correlate with severity in spinal and bulbar muscular atrophy
por: Lombardi, Vittoria, et al.
Publicado: (2019)

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

Muscle: an independent contributor to the neuromuscular spinal muscular atrophy disease phenotype
por: Jha, Narendra N., et al.
Publicado: (2023)

Modeling Spinal Muscular Atrophy in Drosophila
por: Chang, Howard Chia-Hao, et al.
Publicado: (2008)

Carrier screening for spinal muscular atrophy
por: Prior, Thomas W.
Publicado: (2008)

Androgens in spinal and bulbar muscular atrophy
Publicado: (2012)

Spinal Muscular Atrophy: The Treatment Approved
por: Tabet, Rabih, et al.
Publicado: (2017)

Advances in spinal muscular atrophy therapeutics
por: Parente, Valeria, et al.
Publicado: (2018)

Euglycemic Ketoacidosis in Spinal Muscular Atrophy
por: Stoimenis, Dimitrios, et al.
Publicado: (2019)

Metabolic Dysfunction in Spinal Muscular Atrophy
por: Deguise, Marc-Olivier, et al.
Publicado: (2021)

Update on Biomarkers in Spinal Muscular Atrophy
por: Pino, Megan G, et al.
Publicado: (2021)

Onasemnogene abeparvovec for spinal muscular atrophy
Publicado: (2022)

Mitochondrial Dysfunction in Spinal Muscular Atrophy
por: Zilio, Eleonora, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_7h99krtsmecctlcugtfcm4psir