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

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

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

http://www.wikidata.org/entity/Q39521045

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Modeling spinal muscular atrophy in Drosophila Drug treatment for spinal muscular atrophy types II and III Drug treatment for spinal muscular atrophy type I Drug treatment for spinal muscular atrophy types II and III Drug treatment for spinal muscular atrophy type I Drug treatment for spinal muscular atrophy type I Drug treatment for spinal muscular atrophy types II and III A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice Is spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications?Spinal muscular atrophy: development and implementation of potential treatments Molecular, genetic and stem cell-mediated therapeutic strategies for spinal muscular atrophy (SMA)Skeletal muscle DNA damage precedes spinal motor neuron DNA damage in a mouse model of Spinal Muscular Atrophy (SMA)Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway.SAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophy Early heart failure in the SMNDelta7 model of spinal muscular atrophy and correction by postnatal scAAV9-SMN delivery Sustained improvement of spinal muscular atrophy mice treated with trichostatin A plus nutrition Cardiac defects contribute to the pathology of spinal muscular atrophy models Rescue of a severe mouse model for spinal muscular atrophy by U7 snRNA-mediated splicing modulation Effects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophy CNS-targeted gene therapy improves survival and motor function in a mouse model of spinal muscular atrophy.SMN is required for sensory-motor circuit function in Drosophila.Pathological impact of SMN2 mis-splicing in adult SMA mice Protective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy.Somatic Therapy of a Mouse SMA Model with a U7 snRNA Gene Correcting SMN2 Splicing Trans-splicing-mediated improvement in a severe mouse model of spinal muscular atrophy.Effect of diet on the survival and phenotype of a mouse model for spinal muscular atrophy Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?Spinal muscular atrophy: new and emerging insights from model mice Spinal muscular atrophy: mechanisms and therapeutic strategies.Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model Invertebrate models of spinal muscular atrophy: insights into mechanisms and potential therapeutics.Survival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics.Transcriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cells 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 Analysis of the fibroblast growth factor system reveals alterations in a mouse model of spinal muscular atrophy Drug discovery and development for spinal muscular atrophy: lessons from screening approaches and future challenges for clinical development.Temporal requirement for high SMN expression in SMA mice Systemic gene delivery in large species for targeting spinal cord, brain, and peripheral tissues for pediatric disorders A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse Antisense oligonucleotides for the treatment of spinal muscular atrophy.

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P2860

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

http://www.wikidata.org/entity/Q39521045

description

2008 nî lūn-bûn

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2008年の論文

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2008年論文

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Neuronal SMN expression correc ...... sion has no phenotypic effect.

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Neuronal SMN expression correc ...... sion has no phenotypic effect.

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type

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Neuronal SMN expression correc ...... sion has no phenotypic effect.

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Neuronal SMN expression correc ...... sion has no phenotypic effect.

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Neuronal SMN expression correc ...... sion has no phenotypic effect.

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Neuronal SMN expression correc ...... sion has no phenotypic effect.

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Human Molecular Genetics

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Neuronal SMN expression correc ...... sion has no phenotypic effect.

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Arthur H M Burghes

http://www.w3.org/2001/XMLSchema#string

Christine J DiDonato

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Eileen Workman

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Glenn E Morris

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Rocky G Gogliotti

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Tatiana O Gavrilina

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Thomas O Crawford

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Umrao R Monani

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Vicki L McGovern

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P2860

A novel nuclear structure containing the survival of motor neurons protein

Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons?

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

SMN-mediated assembly of RNPs: a complex story

Identification and characterization of a spinal muscular atrophy-determining gene

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

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

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

Dilated cardiomyopathy in the nmd mouse: transgenic rescue and QTLs that improve cardiac function and survival.

Transgenic rescue of neurogenic atrophy in the nmd mouse reveals a role for Ighmbp2 in dilated cardiomyopathy.

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1063-1075

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10.1093/HMG/DDM379

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8

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17

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5670923

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18178576

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spinal muscular atrophy

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2835541

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