Detection of human survival motor neuron (SMN) protein in mice containing the SMN2 transgene: applicability to preclinical therapy development for spinal muscular atrophy

scientific article published on 15 August 2008

Detection of human survival motor neuron (SMN) protein in mice containing the SMN2 transgene: applicability to preclinical therapy development for spinal muscular atrophy is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1016/J.JNEUMETH.2008.07.024
P932PMC publication ID2613335
P698PubMed publication ID18771690
P5875ResearchGate publication ID23237814

P2093author name stringChristian L Lorson
Virginia B Mattis
Matthew E R Butchbach
P2860cites workIndoprofen upregulates the survival motor neuron protein through a cyclooxygenase-independent mechanismQ24613087
Phenylbutyrate increases SMN expression in vitro: relevance for treatment of spinal muscular atrophyQ28177632
Valproic acid increases the SMN2 protein level: a well-known drug as a potential therapy for spinal muscular atrophyQ28201294
An in vivo reporter system for measuring increased inclusion of exon 7 in SMN2 mRNA: potential therapy of SMAQ28204816
Aclarubicin treatment restores SMN levels to cells derived from type I spinal muscular atrophy patientsQ28209134
The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cellsQ28242279
Induction of full-length survival motor neuron by polyphenol botanical compoundsQ28254983
Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblastsQ28261807
Hydroxyurea enhances SMN2 gene expression in spinal muscular atrophy cellsQ28264072
Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophyQ28289793
Identification and characterization of a spinal muscular atrophy-determining geneQ29547495
A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophyQ29617367
A novel method for oral delivery of drug compounds to the neonatal SMNDelta7 mouse model of spinal muscular atrophyQ30488346
A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2.Q33864982
Treatment of spinal muscular atrophy by sodium butyrateQ33933841
Animal models of spinal muscular atrophyQ34045913
Quantitative analyses of SMN1 and SMN2 based on real-time lightCycler PCR: fast and highly reliable carrier testing and prediction of severity of spinal muscular atrophyQ34353103
The survival motor neuron protein in spinal muscular atrophyQ34435656
Identification of proximal spinal muscular atrophy carriers and patients by analysis of SMNT and SMNC gene copy numberQ35250646
Identification of a novel cyclic AMP-response element (CRE-II) and the role of CREB-1 in the cAMP-induced expression of the survival motor neuron (SMN) geneQ35567374
Interferons and IRF-1 induce expression of the survival motor neuron (SMN) genes.Q35943896
A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy.Q36323111
Spinal muscular atrophy: a deficiency in a ubiquitous protein; a motor neuron-specific diseaseQ36347017
Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryosQ36578997
Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophyQ37236684
Protein phosphatase 1 binds to the RNA recognition motif of several splicing factors and regulates alternative pre-mRNA processing.Q38297672
Diverse small-molecule modulators of SMN expression found by high-throughput compound screening: early leads towards a therapeutic for spinal muscular atrophy.Q38325235
Synthesis and biological evaluation of novel 2,4-diaminoquinazoline derivatives as SMN2 promoter activators for the potential treatment of spinal muscular atrophy.Q40022982
Valproic acid increases SMN levels in spinal muscular atrophy patient cells.Q40620622
Correlation between severity and SMN protein level in spinal muscular atrophyQ41102726
The neurobiology of childhood spinal muscular atrophy.Q41489423
The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophyQ43569750
A non-sequence-specific requirement for SMN protein activity: the role of aminoglycosides in inducing elevated SMN protein levelsQ46404171
In vitro and ex vivo evaluation of second-generation histone deacetylase inhibitors for the treatment of spinal muscular atrophyQ48485610
International SMA consortium meeting. (26-28 June 1992, Bonn, Germany)Q67481178
Animal models of spinal muscular atrophyQ80970001
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 SMNQ81385781
P433issue1
P921main subjectspinal muscular atrophyQ580290
P304page(s)36-43
P577publication date2008-08-15
P1433published inJournal of Neuroscience MethodsQ15764020
P1476titleDetection of human survival motor neuron (SMN) protein in mice containing the SMN2 transgene: applicability to preclinical therapy development for spinal muscular atrophy
P478volume175

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cites work (P2860)
Q39350652A novel morpholino oligomer targeting ISS-N1 improves rescue of severe spinal muscular atrophy transgenic mice
Q36079719Antisense-mediated exon inclusion
Q34082155Assays for the identification and prioritization of drug candidates for spinal muscular atrophy
Q30492560Effects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophy
Q34760255Enhancement of SMN protein levels in a mouse model of spinal muscular atrophy using novel drug-like compounds
Q39549155Generation of a tamoxifen inducible SMN mouse for temporal SMN replacement
Q34123129Identification of novel compounds that increase SMN protein levels using an improved SMN2 reporter cell assay.
Q38006384Mouse models of SMA: tools for disease characterization and therapeutic development
Q34065993Splicing of the Survival Motor Neuron genes and implications for treatment of SMA
Q41683448Systems biology investigation of cAMP modulation to increase SMN levels for the treatment of spinal muscular atrophy

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