(Q37875033)

24 August 2017

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title

Synaptic defects in spinal muscular atrophy animal models (English)

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24 August 2017

main subject

spinal muscular atrophy

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1 reference

Rocío Ruiz

2

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Lucia Tabares

object named as

Lucía Tabares

series ordinal

3

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author name string

Laura Torres-Benito

series ordinal

1

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24 August 2017

publication date

1 January 2012

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24 August 2017

number of pages

8

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Developmental Neurobiology

inferred from page(s)

published in

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24 August 2017

volume

72

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24 August 2017

issue

1

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24 August 2017

page(s)

126-133

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Mitochondrial dysfunction in a neural cell model of spinal muscular atrophy.

24 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Colocalization of synapsin and actin during synaptic vesicle recycling

21 January 2018

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SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesis.

21 January 2018

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Deletion of smn-1, the Caenorhabditis elegans ortholog of the spinal muscular atrophy gene, results in locomotor dysfunction and reduced lifespan

21 January 2018

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

21 January 2018

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Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis.

21 January 2018

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The neurobiology of childhood spinal muscular atrophy.

21 January 2018

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Neuromuscular defects in a Drosophila survival motor neuron gene mutant.

21 January 2018

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https://api.crossref.org/works/10.1002%2FDNEU.20912

The role of RNP biogenesis in spinal muscular atrophy

21 January 2018

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https://api.crossref.org/works/10.1002%2FDNEU.20912

RhoA/ROCK regulation of neuritogenesis via profilin IIa-mediated control of actin stability

21 January 2018

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https://api.crossref.org/works/10.1002%2FDNEU.20912

Survival motor neuron (SMN) protein: role in neurite outgrowth and neuromuscular maturation during neuronal differentiation and development.

21 January 2018

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https://api.crossref.org/works/10.1002%2FDNEU.20912

The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis

21 January 2018

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Functional genomic analysis of C. elegans chromosome I by systematic RNA interference

21 January 2018

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https://api.crossref.org/works/10.1002%2FDNEU.20912

Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Survival motor neuron gene transcript analysis in muscles from spinal muscular atrophy patients.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

The heterogeneous nuclear ribonucleoprotein-R is necessary for axonal beta-actin mRNA translocation in spinal motor neurons

21 January 2018

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Generation and Characterization of a genetic zebrafish model of SMA carrying the human SMN2 gene.

21 January 2018

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Mitochondrial dysfunction and amyotrophic lateral sclerosis

21 January 2018

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Co-regulation of survival of motor neuron (SMN) protein and its interactor SIP1 during development and in spinal muscular atrophy

21 January 2018

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Defective Ca2+ channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

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

21 January 2018

1 reference

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

21 January 2018

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Identification and characterization of a spinal muscular atrophy-determining gene

21 January 2018

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Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophy

21 January 2018

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https://api.crossref.org/works/10.1002%2FDNEU.20912

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

21 January 2018

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Embryonic motor axon development in the severe SMA mouse

21 January 2018

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Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding.

21 January 2018

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https://api.crossref.org/works/10.1002%2FDNEU.20912

A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPs

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

The Caenorhabditis elegans orthologue of the human gene responsible for spinal muscular atrophy is a maternal product critical for germline maturation and embryonic viability.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Regulation of mitochondria distribution by RhoA and formins

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

The essential role of the mitochondria-dependent death-signaling cascade in chemotherapy-induced potentiation of Apo2L/TRAIL cytotoxicity in cultured thoracic cancer cells: amplified caspase 8 is indispensable for combination-mediated massive cell d

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Plastin 3 is a protective modifier of autosomal recessive spinal muscular atrophy

21 January 2018

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https://api.crossref.org/works/10.1002%2FDNEU.20912

Chaperoning ribonucleoprotein biogenesis in health and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Essential role for the SMN complex in the specificity of snRNP assembly

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

The COPI vesicle complex binds and moves with survival motor neuron within axons

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Development of the vertebrate neuromuscular junction

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Impaired recycling of synaptic vesicles after acute perturbation of the presynaptic actin cytoskeleton

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Full-genome RNAi profiling of early embryogenesis in Caenorhabditis elegans

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Reduced levels of survival motor neuron protein leads to aberrant motoneuron growth in a Xenopus model of muscular atrophy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Multiprotein complexes of the survival of motor neuron protein SMN with Gemins traffic to neuronal processes and growth cones of motor neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

Smn depletion alters profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrity

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

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/10.1002%2FDNEU.20912

7 January 2021

Spinal muscular atrophy: the RNP connection

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

7 January 2021

An FCCP-sensitive Ca2+ store in bullfrog sympathetic neurons and its participation in stimulus-evoked changes in [Ca2+]i.

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

7 January 2021

A new model to study spinal muscular atrophy: Neurite degeneration and cell death is counteracted by BCL-XL Overexpression in motoneurons

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

7 January 2021

Subcellular localization and axonal transport of the survival motor neuron (SMN) protein in the developing rat spinal cord

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

7 January 2021

Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization.

1 reference

https://api.crossref.org/works/10.1002%2FDNEU.20912

7 January 2021

Identifiers

DOI

10.1002/DNEU.20912

1 reference

24 August 2017

1 reference