Neuromuscular defects and breathing disorders in a new mouse model of spinal muscular atrophy.
about
The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophyCopy Number Variations in the Survival Motor Neuron Genes: Implications for Spinal Muscular Atrophy and Other Neurodegenerative DiseasesSynaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophyProtective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy.Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpainThe effect of diet on the protective action of D156844 observed in spinal muscular atrophy mice.Splicing of the Survival Motor Neuron genes and implications for treatment of SMATranscriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cellsDrug discovery and development for spinal muscular atrophy: lessons from screening approaches and future challenges for clinical development.A humanized Smn gene containing the SMN2 nucleotide alteration in exon 7 mimics SMN2 splicing and the SMA disease phenotype.Therapeutic strategies for the treatment of spinal muscular atrophy.Inhibition of apoptosis blocks human motor neuron cell death in a stem cell model of spinal muscular atrophy.Enhancement of SMN protein levels in a mouse model of spinal muscular atrophy using novel drug-like compoundsSMN deficiency alters Nrxn2 expression and splicing in zebrafish and mouse models of spinal muscular atrophy.Spinal muscular atrophy: journeying from bench to bedsideDisruption of the Survival Motor Neuron (SMN) gene in pigs using ssDNA.SMN1 and SMN2 copy numbers in cell lines derived from patients with spinal muscular atrophy as measured by array digital PCRThe effect of the DcpS inhibitor D156844 on the protective action of follistatin in mice with spinal muscular atrophy.Failure of lower motor neuron radial outgrowth precedes retrograde degeneration in a feline model of spinal muscular atrophy.Behavioral and electrophysiological outcomes of tissue-specific Smn knockdown in Drosophila melanogaster.Spinal muscular atrophy: an update on therapeutic progressMore than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases.Gene activation of SMN by selective disruption of lncRNA-mediated recruitment of PRC2 for the treatment of spinal muscular atrophy.Mouse models of SMA: tools for disease characterization and therapeutic development.Developments in the discovery of drugs for spinal muscular atrophy: successful beginnings and future prospects.In vitro neurogenesis: development and functional implications of iPSC technology.Diverse role of survival motor neuron protein.How the discovery of ISS-N1 led to the first medical therapy for spinal muscular atrophyHypoxia is a modifier of SMN2 splicing and disease severity in a severe SMA mouse modelThe effects of C5-substituted 2,4-diaminoquinazolines on selected transcript expression in spinal muscular atrophy cellsSystems biology investigation of cAMP modulation to increase SMN levels for the treatment of spinal muscular atrophyPhysical exercise reduces cardiac defects in type 2 spinal muscular atrophy-like mice.Optimizing mouse models of neurodegenerative disorders: are therapeutics in sight?
P2860
Q24604444-2130DE51-AF3A-4AE0-9BA2-979425FE9709Q26750803-62728315-081D-433F-A286-ABB53FC7703EQ27312456-CBFB8268-7F12-4537-AB28-1A2BCDC5CB97Q30743508-A2D735AF-9558-43ED-B34F-B221965CA8D9Q30994078-B365E764-80B6-4351-AAB4-001D3920E435Q33645966-80A3C759-4FD6-4BBE-8A05-8A523AC80E9DQ34065993-3F0427FD-9011-4A06-BE07-6364CC6E25EAQ34142599-FD272A70-C44A-4565-BB9E-DC2080BEC795Q34172502-04128F60-BBB6-4DE2-BAAE-0EE0B03C62EEQ34184793-8BD1CE59-7D29-49B2-9AF8-8B38B0CE07BBQ34296140-CB25182D-8223-472D-B09D-C37782F053C6Q34314191-AD8641A2-9487-437A-9CD9-2B22038A56A0Q34760255-E9528A55-C588-4E74-B3F9-7E14DB025EADQ35039158-C6268EB6-CCFA-4B2A-AC1B-53285892063EQ35340133-FEBFF6A3-9578-4874-8939-7F017D4BFB95Q35683524-F712489B-3202-44CE-8AC7-2272102343E7Q35907736-688CE166-85D7-4C15-AD8D-3B5641A9F3C7Q35967715-F073236C-5BEC-411B-A9DF-DF6E229D640EQ35968791-DBDC4266-69CA-4027-9A62-62D4D3F8BFD4Q36409666-988D9232-3AD3-445E-90B5-E4362D5C0AF1Q37302832-C7671F6B-1382-4691-9E92-EE673719E8A6Q37400655-1703C721-B18A-4548-93BB-8527EF576589Q37682281-843EF56B-9A29-433D-86A5-0D1689F179ADQ38006384-B7891DCA-FA3B-499E-A6CB-240B20E3F76AQ38014955-188C9697-03F7-49C7-99CA-0C16440EC0F4Q38164195-7E8B5923-5478-401E-82BA-19E4AD5259F9Q39092025-624E8181-15A3-4147-9186-47C1BE16E63EQ39293468-B5DF8BCD-7E35-4021-BD76-86A60E719789Q39319736-5D1C6C55-48D2-43B9-85B3-97D7A44E70A6Q41000953-225ABA29-3DCA-4D2D-8CA0-7824DB81E1ABQ41683448-97DE9D64-08EB-4C50-8969-34B8D8A51B28Q44414821-E5ADAD37-67AF-4EEC-868C-C59C94163194Q55140067-DDABD87C-C526-42F9-8940-92173D54CD94
P2860
Neuromuscular defects and breathing disorders in a new mouse model of spinal muscular atrophy.
description
2010 nî lūn-bûn
@nan
2010年の論文
@ja
2010年学术文章
@wuu
2010年学术文章
@zh
2010年学术文章
@zh-cn
2010年学术文章
@zh-hans
2010年学术文章
@zh-my
2010年学术文章
@zh-sg
2010年學術文章
@yue
2010年學術文章
@zh-hant
name
Neuromuscular defects and brea ...... el of spinal muscular atrophy.
@en
Neuromuscular defects and brea ...... el of spinal muscular atrophy.
@nl
type
label
Neuromuscular defects and brea ...... el of spinal muscular atrophy.
@en
Neuromuscular defects and brea ...... el of spinal muscular atrophy.
@nl
prefLabel
Neuromuscular defects and brea ...... el of spinal muscular atrophy.
@en
Neuromuscular defects and brea ...... el of spinal muscular atrophy.
@nl
P2093
P50
P1476
Neuromuscular defects and brea ...... el of spinal muscular atrophy.
@en
P2093
Brunhilde Wirth
Emile Andriambeloson
Estelle Durand
Fabrice Robert
Jorge Gallego
Magali Michaud
Marc Giraudon-Paoli
Rebecca M Pruss
Serena Bielli
Sibylle Jablonka
P304
P356
10.1016/J.NBD.2010.01.006
P577
2010-01-18T00:00:00Z