Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy
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Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophyModeling spinal muscular atrophy in DrosophilaDrug treatment for spinal muscular atrophy types II and IIIDrug treatment for spinal muscular atrophy type IDrug treatment for spinal muscular atrophy types II and IIIDrug treatment for spinal muscular atrophy type IThe zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy.The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophyNeuromuscular Junctions as Key Contributors and Therapeutic Targets in Spinal Muscular AtrophyIs spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications?Skeletal muscle DNA damage precedes spinal motor neuron DNA damage in a mouse model of Spinal Muscular Atrophy (SMA)Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophySAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophySelective Neuromuscular Denervation in Taiwanese Severe SMA Mouse Can Be Reversed by Morpholino Antisense OligonucleotidesEffects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophyCNS-targeted gene therapy improves survival and motor function in a mouse model of spinal muscular atrophy.Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy.Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophySMN is required for sensory-motor circuit function in Drosophila.Temporal requirement for SMN in motoneuron development.The DcpS inhibitor RG3039 improves survival, function and motor unit pathologies in two SMA mouse models.Pathological impact of SMN2 mis-splicing in adult SMA miceRequirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation.Pharmacokinetics, pharmacodynamics, and efficacy of a small-molecule SMN2 splicing modifier in mouse models of spinal muscular atrophyBrain microvasculature defects and Glut1 deficiency syndrome averted by early repletion of the glucose transporter-1 protein.Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpainEffect of genetic background on the phenotype of the Smn2B/- mouse model of spinal muscular atrophy.Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy.Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: in vitro versus in vivo developmentInflammation in ALS and SMA: sorting out the good from the evil.Six-Minute Walk Test demonstrates motor fatigue in spinal muscular atrophyConserved genes act as modifiers of invertebrate SMN loss of function defectsSpinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?Spinal muscular atrophy: new and emerging insights from model miceProteomic assessment of a cell model of spinal muscular atrophyMotor unit abnormalities in Dystonia musculorum miceDefining the therapeutic window in a severe animal model of spinal muscular atrophySMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminalsSurvival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics.Transcriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cells
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Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
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bilimsel makale
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scientific article published on 20 May 2008
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Reduced SMN protein impairs ma ...... els of spinal muscular atrophy
@en
Reduced SMN protein impairs ma ...... ls of spinal muscular atrophy.
@nl
type
label
Reduced SMN protein impairs ma ...... els of spinal muscular atrophy
@en
Reduced SMN protein impairs ma ...... ls of spinal muscular atrophy.
@nl
prefLabel
Reduced SMN protein impairs ma ...... els of spinal muscular atrophy
@en
Reduced SMN protein impairs ma ...... ls of spinal muscular atrophy.
@nl
P2093
P2860
P356
P1476
Reduced SMN protein impairs ma ...... els of spinal muscular atrophy
@en
P2093
Cathleen Lutz
Gyu-Hwan Park
Lynn T Landmesser
Marc S Arkovitz
Olga Leykekhman
Shingo Kariya
Umrao R Monani
Yuka Maeno-Hikichi
P2860
P304
P356
10.1093/HMG/DDN156
P577
2008-05-20T00:00:00Z