(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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scholarly article

1 reference

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

1 reference

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

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

language of work or name

0 references

publication date

30 May 2011

1 reference

Europe PubMed Central

PubMed publication ID

6 August 2017

Developmental Biology

1 reference

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

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

28 July 2018

Synaptic defects in the spinal and neuromuscular circuitry 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

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

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

28 July 2018

Cardiac defects contribute to the pathology of spinal muscular atrophy models

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

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

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: new and emerging insights from model mice

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

RETRACTED ARTICLE: Rescue of the spinal muscular atrophy phenotype in a mouse model by early postnatal delivery of SMN

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

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

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

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

Impaired synaptic vesicle release and immaturity of neuromuscular junctions in 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

28 July 2018

Embryonic motor axon development in the severe SMA mouse

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

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

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 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

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

28 July 2018

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

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

Identification and characterization of a spinal muscular atrophy-determining gene

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 size and motor unit survival in mice

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

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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