(Q40235775)

18 September 2017

title

SMN deficiency does not induce oxidative stress in SMA iPSC-derived astrocytes or motor neurons (English)

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18 September 2017

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Teresa N Patitucci

1

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18 September 2017

Allison D Ebert

series ordinal

2

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18 September 2017

publication date

7 December 2015

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18 September 2017

published in

Human Molecular Genetics

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18 September 2017

volume

25

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18 September 2017

issue

3

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18 September 2017

page(s)

514-523

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Pan-ethnic carrier screening and prenatal diagnosis for spinal muscular atrophy: clinical laboratory analysis of >72,400 specimens

18 September 2017

cites work

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Identification and characterization of a spinal muscular atrophy-determining gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Antisense oligonucleotides for the treatment of spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Intravenous scAAV9 delivery of a codon-optimized SMN1 sequence rescues SMA mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Intravenous administration of self-complementary AAV9 enables transgene delivery to adult motor neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

RETRACTED ARTICLE: Rescue of the spinal muscular atrophy phenotype in a mouse model by early postnatal delivery of SMN

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Decreasing disease severity in symptomatic, Smn(-/-);SMN2(+/+), spinal muscular atrophy mice following scAAV9-SMN delivery

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Systemic gene delivery in large species for targeting spinal cord, brain, and peripheral tissues for pediatric disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Intravascular AAV9 preferentially targets neonatal neurons and adult astrocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Inhibition of apoptosis blocks human motor neuron cell death in a stem cell model of spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Motor neuron rescue in spinal muscular atrophy mice demonstrates that sensory-motor defects are a consequence, not a cause, of motor neuron dysfunction

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Astrocytes influence the severity of spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

GDNF: a potent survival factor for motoneurons present in peripheral nerve and muscle

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Programmed cell death of embryonic motoneurons triggered through the Fas death receptor

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Neuropathological analysis in spinal muscular atrophy type II.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Neonatal spinal muscular atrophy with multiple contractures, bone fractures, respiratory insufficiency and 5q13 deletion.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Spinal muscular atrophy astrocytes exhibit abnormal calcium regulation and reduced growth factor production

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Mitochondria, oxidative stress and neurodegeneration.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

NAD(P)H oxidase and uncoupled nitric oxide synthase are major sources of glomerular superoxide in rats with experimental diabetic nephropathy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Imbalance between xanthine oxidase and nitric oxide synthase signaling pathways underlies mechanoenergetic uncoupling in the failing heart

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Partial restoration of cardio-vascular defects in a rescued severe model of spinal muscular atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Recapitulation of spinal motor neuron-specific disease phenotypes in a human cell model of spinal muscular atrophy.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Necroptosis drives motor neuron death in models of both sporadic and familial ALS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Brief report: phenotypic rescue of induced pluripotent stem cell-derived motoneurons of a spinal muscular atrophy patient.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Quantitative microplate-based respirometry with correction for oxygen diffusion

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Oxidative DNA damage estimated by 8-hydroxydeoxyguanosine excretion in humans: influence of smoking, gender and body mass index

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

SMN is required for sensory-motor circuit function in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Survival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

The role of mitochondria in inherited neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Mitochondria-targeted catalase reverts the neurotoxicity of hSOD1G⁹³A astrocytes without extending the survival of ALS-linked mutant hSOD1 mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

A new method for the rapid and long term growth of human neural precursor cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

EZ spheres: a stable and expandable culture system for the generation of pre-rosette multipotent stem cells from human ESCs and iPSCs

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

Defective Neuromuscular Junction Organization and Postnatal Myogenesis in Mice With Severe Spinal Muscular Atrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDV489

21 January 2018

Identifiers

DOI

10.1093/HMG/DDV489

1 reference

18 September 2017

1 reference