(Q35126847)

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Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons

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Europe PubMed Central

PubMed publication ID

6 August 2017

Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons (English)

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

spinal muscular atrophy

1 reference

based on heuristic

inferred from title

muscular atrophy

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based on heuristic

inferred from title

author name string

Young Il Lee

1

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

Michelle Mikesh

2

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

Ian Smith

3

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

Mendell Rimer

4

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

Wesley Thompson

5

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Europe PubMed Central

PubMed publication ID

6 August 2017

language of work or name

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publication date

30 May 2011

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Europe PubMed Central

PubMed publication ID

6 August 2017

Developmental Biology

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Europe PubMed Central

PubMed publication ID

6 August 2017

356

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

2

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

432-444

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

Early functional impairment of sensory-motor connectivity in a mouse model of spinal muscular atrophy

1 reference

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28 July 2018

Synaptic Defects in the Spinal and Neuromuscular Circuitry in a Mouse Model of Spinal Muscular Atrophy

1 reference

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Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene

1 reference

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28 July 2018

Cardiac defects contribute to the pathology of spinal muscular atrophy models

1 reference

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28 July 2018

Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice

1 reference

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28 July 2018

Spinal muscular atrophy: new and emerging insights from model mice

1 reference

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28 July 2018

RETRACTED ARTICLE: Rescue of the spinal muscular atrophy phenotype in a mouse model by early postnatal delivery of SMN

1 reference

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28 July 2018

Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?

1 reference

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28 July 2018

Rescuing Z+ agrin splicing in Nova null mice restores synapse formation and unmasks a physiologic defect in motor neuron firing

1 reference

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28 July 2018

Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice

1 reference

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28 July 2018

Embryonic motor axon development in the severe SMA mouse

1 reference

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28 July 2018

Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy

1 reference

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28 July 2018

Spinal muscular atrophy: a deficiency in a ubiquitous protein; a motor neuron-specific disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Spinal muscular atrophy: a delayed development hypothesis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Thrombospondins are astrocyte-secreted proteins that promote CNS synaptogenesis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Lentivector-mediated SMN replacement in a mouse model of spinal muscular atrophy.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Fluorescent proteins expressed in mouse transgenic lines mark subsets of glia, neurons, macrophages, and dendritic cells for vital examination.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Deletion of murine Smn exon 7 directed to liver leads to severe defect of liver development associated with iron overload

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Nerve-independent formation of a topologically complex postsynaptic apparatus.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Terminal Schwann cells guide the reinnervation of muscle after nerve injury

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Caveolinopathies: mutations in caveolin-3 cause four distinct autosomal dominant muscle diseases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Roles of neurotransmitter in synapse formation: development of neuromuscular junctions lacking choline acetyltransferase

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Muscle activity and muscle agrin regulate the organization of cytoskeletal proteins and attached acetylcholine receptor (AchR) aggregates in skeletal muscle fibers

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Deletion of murine SMN exon 7 directed to skeletal muscle leads to severe muscular dystrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Absence of alpha-syntrophin leads to structurally aberrant neuromuscular synapses deficient in utrophin

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Nuclear targeting defect of SMN lacking the C-terminus in a mouse model of spinal muscular atrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

A mouse model for spinal muscular atrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Electron microscopic study of long-term denervated rat skeletal muscle

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

The neurobiology of childhood spinal muscular atrophy.

1 reference

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28 July 2018

Aberrant differentiation of neuromuscular junctions in mice lacking s-laminin/laminin beta 2

1 reference

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28 July 2018

Identification and characterization of a spinal muscular atrophy-determining gene

1 reference

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28 July 2018

Muscle size and motor unit survival in mice

1 reference

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28 July 2018

An experimental study of acute neonatal enterocolitis--the importance of breast milk

1 reference

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28 July 2018

Control of end-plate channel properties by neurotrophic effects and by muscle activity in rat.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

The effect of prolonged, reversible block of nerve impulses on the elimination of polyneuronal innervation of new-born rat skeletal muscle fibers

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

28 July 2018

Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of spinal muscular atrophy.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

SMN deficiency disrupts brain development in a mouse model of severe spinal muscular atrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Altered intracellular Ca2+ homeostasis in nerve terminals of severe spinal muscular atrophy mice.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

The developmental pattern of myotubes in spinal muscular atrophy indicates prenatal delay of muscle maturation.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Childhood spinal muscular atrophy induces alterations in contractile and regulatory protein isoform expressions.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Exercise-induced activation of NMDA receptor promotes motor unit development and survival in a type 2 spinal muscular atrophy model mouse.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Regulation of the intermediate filament protein nestin at rodent neuromuscular junctions by innervation and activity.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Induction of neuregulin signaling in mouse schwann cells in vivo mimics responses to denervation.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Reconstruct: a free editor for serial section microscopy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Glial cells maintain synaptic structure and function and promote development of the neuromuscular junction in vivo

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Domain IV of mouse laminin beta1 and beta2 chains

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Intragenic telSMN mutations: frequency, distribution, evidence of a founder effect, and modification of the spinal muscular atrophy phenotype by cenSMN copy number.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

gamma-AChR/epsilon-AChR switch at agrin-induced postsynaptic-like apparatus in skeletal muscle

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Agrin-induced postsynaptic-like apparatus in skeletal muscle fibers in vivo

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Electrical activity-dependent regulation of the acetylcholine receptor delta-subunit gene, MyoD, and myogenin in primary myotubes

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3143211

27 September 2018

Degradation of two AChR populations at rat neuromuscular junctions: regulation in vivo by electrical stimulation

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse model

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

Development of muscle fiber types in the prenatal rat hindlimb

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

Differentiation of fiber types in aneural musculature of the prenatal rat hindlimb

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

Reduced survival motor neuron (Smn) gene dose in mice leads to motor neuron degeneration: an animal model for spinal muscular atrophy type III

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

Schwann cells proliferate at rat neuromuscular junctions during development and regeneration

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

From plaque to pretzel: fold formation and acetylcholine receptor loss at the developing neuromuscular junction

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

A synaptic laminin–calcium channel interaction organizes active zones in motor nerve terminals

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

Synapse elimination in neonatal rat muscle is sensitive to pattern of muscle use

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

Lack of segmental selectivity in elimination of synapses from soleus muscle of new-born rats

1 reference

https://api.crossref.org/works/10.1016%2FJ.YDBIO.2011.05.667

7 January 2021

Identifiers

10.1016/J.YDBIO.2011.05.667

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

PMC publication ID

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

ResearchGate publication ID

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