(Q35064279)

6 August 2017

title

Selective vulnerability of spinal and cortical motor neuron subpopulations in delta7 SMA mice (English)

1 reference

6 August 2017

Denise Locatelli

6

0 references

Antonio Piras

Antonio Piras

3

0 references

Valeria Valsecchi

Valeria Valsecchi

4

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Marina Boido

Marina Boido

2

0 references

Alessandro Vercelli

Alessandro Vercelli

9

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author name string

Paolo d'Errico

1

1 reference

6 August 2017

Elena De Amicis

5

1 reference

6 August 2017

Silvia Capra

7

1 reference

6 August 2017

Francesco Vagni

8

1 reference

6 August 2017

Giorgio Battaglia

10

1 reference

6 August 2017

language of work or name

English

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publication date

6 December 2013

1 reference

6 August 2017

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6 August 2017

volume

8

1 reference

6 August 2017

issue

12

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6 August 2017

page(s)

e82654

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Creative Commons Attribution 4.0 International

6 August 2017

copyright license

start time

6 December 2013

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

describes a project that uses

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3855775/fullTextXML

inferred from PubMed Central ID database lookup

cites work

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Peripheral SMN restoration is essential for long-term rescue of a severe spinal muscular atrophy mouse model

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8 July 2018

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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

8 July 2018

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8 July 2018

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

8 July 2018

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Early heart failure in the SMNDelta7 model of spinal muscular atrophy and correction by postnatal scAAV9-SMN delivery

8 July 2018

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CNS-targeted gene therapy improves survival and motor function in a mouse model of spinal muscular atrophy

8 July 2018

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8 July 2018

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Long-term potentiation depends on release of D-serine from astrocytes

8 July 2018

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Inflammation in ALS and SMA: sorting out the good from the evil

8 July 2018

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A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice

8 July 2018

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

8 July 2018

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Congenital heart disease is a feature of severe infantile spinal muscular atrophy

8 July 2018

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Embryonic motor axon development in the severe SMA mouse

8 July 2018

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Restoring Bcl-x(L) levels benefits a mouse model of spinal muscular atrophy

8 July 2018

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

8 July 2018

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

8 July 2018

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Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy

8 July 2018

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Axonal-SMN (a-SMN), a protein isoform of the survival motor neuron gene, is specifically involved in axonogenesis.

8 July 2018

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8 July 2018

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8 July 2018

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

8 July 2018

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A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy.

8 July 2018

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Regulation of murine survival motor neuron (Smn) protein levels by modifying Smn exon 7 splicing

8 July 2018

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The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophy

8 July 2018

1 reference

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A mouse model for spinal muscular atrophy

8 July 2018

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8 July 2018

1 reference

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

8 July 2018

1 reference

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A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing

8 July 2018

1 reference

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BDNF and NT-4/5 prevent atrophy of rat rubrospinal neurons after cervical axotomy, stimulate GAP-43 and Talpha1-tubulin mRNA expression, and promote axonal regeneration

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3855775

Expression of the SMN gene, the spinal muscular atrophy determining gene, in the mammalian central nervous system

8 July 2018

1 reference

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

8 July 2018

1 reference

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Identification and characterization of a spinal muscular atrophy-determining gene

8 July 2018

1 reference

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

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3855775

Unbiased stereological estimation of the total number of neurons in the subdivisions of the rat hippocampus using the optical fractionator

8 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3855775

Neuronal death is enhanced and begins during foetal development in type I spinal muscular atrophy spinal cord

8 July 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

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/10.1371%2FJOURNAL.PONE.0082654

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

Downregulation of Bcl-2 proteins in type I spinal muscular atrophy motor neurons during fetal development.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

Cell death and decreased synaptic protein expression in the ventral horn of Holstein-Friesian calves with spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

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/10.1371%2FJOURNAL.PONE.0082654

Adult spinal muscular atrophy. A report of four cases

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

A unilateral section of the corticospinal tract at cervical level in primate does not lead to measurable cell loss in motor cortex

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

Astroglial metabolic networks sustain hippocampal synaptic transmission

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0082654

Influence of the axotomy to cell body distance in rat rubrospinal and spinal motoneurons: differential regulation of GAP-43, tubulins, and neurofilament-M

21 January 2018

1 reference

12 December 2020

Detection of the survival motor neuron (SMN) genes by FISH: further evidence for a role for SMN2 in the modulation of disease severity in SMA patients

1 reference

12 December 2020

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

1 reference

12 December 2020

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

12 December 2020

Ultrastructural analysis and TUNEL demonstrate motor neuron apoptosis in Werdnig-Hoffmann disease

1 reference

12 December 2020

An intrinsic distinction in neuromuscular junction assembly and maintenance in different skeletal muscles

1 reference

12 December 2020

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

12 December 2020

Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy

1 reference

12 December 2020

Human mesenchymal stem cell transplantation extends survival, improves motor performance and decreases neuroinflammation in mouse model of amyotrophic lateral sclerosis

1 reference

12 December 2020

Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice

1 reference

12 December 2020

Newborn and carrier screening for spinal muscular atrophy

1 reference

12 December 2020

Therapeutic benefits of cardiotrophin-1 gene transfer in a mouse model of spinal muscular atrophy

1 reference

12 December 2020

10.1371/JOURNAL.PONE.0082654

Identifiers

DOI

1 reference

6 August 2017

0 references

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6 August 2017

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