SMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy

scientific article

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SMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1093/HMG/DDT612
P698PubMed publication ID24301677
P5875ResearchGate publication ID259154831

P50authorThomas H. GillingwaterQ37373419
P2093author name stringGillian Hunter
Sarah L Roche
Arwin Aghamaleky Sarvestany
Rebecca C Symes
P2860cites workSpinal muscular atrophy astrocytes exhibit abnormal calcium regulation and reduced growth factor productionQ37614887
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyondQ37642283
Glial cells in amyotrophic lateral sclerosisQ37902077
Spinal muscular atrophy: going beyond the motor neuronQ38066335
Unwrapping the genes of myelinQ38211942
A mouse model for spinal muscular atrophyQ38316939
Protein zero gene expression is regulated by the glial transcription factor Sox10Q39452670
Defective Ca2+ channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophyQ39751507
Direct regulation of myelin protein zero expression by the Egr2 transactivatorQ40337072
SCIP: a glial POU domain gene regulated by cyclic AMP.Q41836479
Arrest of myelination and reduced axon growth when Schwann cells lack mTOR.Q41880723
P(0) glycoprotein overexpression causes congenital hypomyelination of peripheral nervesQ42919149
The dorsolateral corticospinal tract in mice: an alternative route for corticospinal input to caudal segments following dorsal column lesionsQ43533356
The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophyQ43569750
Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophyQ43932877
A role for motoneuron subtype-selective ER stress in disease manifestations of FALS miceQ46070173
Cytoarchitecture of the Spinal Cord of the Postnatal (P4) MouseQ46563242
Myelination in mouse dorsal root ganglion/Schwann cell coculturesQ46800185
Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophyQ46861362
Mouse schwann cells need both NRG1 and cyclic AMP to myelinateQ47169288
Synaptic defects in type I spinal muscular atrophy in human developmentQ47322638
Peripheral nerve abnormalities in pediatric patients with spinal muscular atrophyQ48562187
Isolation, purification and expansion of myelination-competent, neonatal mouse Schwann cells.Q50929339
Laminin induced local axonal translation of β-actin mRNA is impaired in SMN-deficient motoneuronsQ53151357
Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophyQ21144988
Wrapping it up: the cell biology of myelinationQ22337362
The POU factor Oct-6 and Schwann cell differentiationQ24319829
Glial cells as intrinsic components of non-cell-autonomous neurodegenerative diseaseQ24621854
SAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophyQ28271235
Neurofascins are required to establish axonal domains for saltatory conductionQ28512452
Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneuronsQ28576647
Identification and characterization of a spinal muscular atrophy-determining geneQ29547495
Descending pathways to the spinal cord, III: Sites of origin of the corticospinal tractQ29998971
Analysis of congenital hypomyelinating Egr2Lo/Lo nerves identifies Sox2 as an inhibitor of Schwann cell differentiation and myelinationQ30856297
Spinal muscular atrophy.Q33345749
The molecular machinery of myelin gene transcription in Schwann cellsQ34092185
Prospects for the gene therapy of spinal muscular atrophyQ34165703
Protein composition of myelin of the peripheral nervous systemQ34216787
The origin and development of glial cells in peripheral nervesQ34447586
Morphological characteristics of motor neurons do not determine their relative susceptibility to degeneration in a mouse model of severe spinal muscular atrophyQ34534222
Motor nerve conduction velocity in spinal muscular atrophy of childhoodQ34989452
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 neuronsQ35126847
Disease mechanisms in inherited neuropathiesQ35209953
Dysfunction of axonal membrane conductances in adolescents and young adults with spinal muscular atrophyQ35540059
Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophyQ35605208
Schwann cell myelination requires integration of laminin activitiesQ36407316
Interactions of Sox10 and Egr2 in myelin gene regulationQ37020396
Regulation of Schwann cell function by the extracellular matrix.Q37273622
Molecular domains of myelinated axons in the peripheral nervous systemQ37273627
Cell death in the Schwann cell lineage and its regulation by neuregulinQ37390514
Notch controls embryonic Schwann cell differentiation, postnatal myelination and adult plasticity.Q37436957
P433issue9
P921main subjectSchwann cellQ465621
spinal muscular atrophyQ580290
P304page(s)2235-2250
P577publication date2013-12-02
P1433published inHuman Molecular GeneticsQ2720965
P1476titleSMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy
P478volume23

Reverse relations

cites work (P2860)
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Q39774850Restoration of SMN in Schwann cells reverses myelination defects and improves neuromuscular function in spinal muscular atrophy.
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Q26782551UBA1: At the Crossroads of Ubiquitin Homeostasis and Neurodegeneration