SMN deficiency in severe models of spinal muscular atrophy causes widespread intron retention and DNA damage
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Spinal Muscular Atrophy: From Defective Chaperoning of snRNP Assembly to Neuromuscular Dysfunction.Downregulation of Survivin contributes to cell-cycle arrest during postnatal cardiac development in a severe spinal muscular atrophy mouse model.Converging Mechanisms of p53 Activation Drive Motor Neuron Degeneration in Spinal Muscular Atrophy.RNA-binding proteins in neurodegeneration: mechanisms in aggregate.SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities.Small-molecule flunarizine increases SMN protein in nuclear Cajal bodies and motor function in a mouse model of spinal muscular atrophy.R-loops cause genomic instability in T helper lymphocytes from patients with Wiskott-Aldrich syndrome.Perturbed autophagy and DNA repair converge to promote neurodegeneration in amyotrophic lateral sclerosis and dementia.Recent Advances in Antisense Oligonucleotide Therapy in Genetic Neuromuscular Diseases.Alternative mRNA Splicing in the Pathogenesis of Obesity.Interactome analyses revealed that the U1 snRNP machinery overlaps extensively with the RNAP II machinery and contains multiple ALS/SMA-causative proteins.Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA.Searching the overlap between network modules with specific betweeness (S2B) and its application to cross-disease analysisUnraveling the Pathways to Neuronal Homeostasis and Disease: Mechanistic Insights into the Role of RNA-Binding Proteins and Associated Factors
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SMN deficiency in severe models of spinal muscular atrophy causes widespread intron retention and DNA damage
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article científic
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article scientifique
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articolo scientifico
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artigo científico
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bilimsel makale
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scientific article published on 07 March 2017
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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SMN deficiency in severe model ...... ntron retention and DNA damage
@en
SMN deficiency in severe model ...... tron retention and DNA damage.
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type
label
SMN deficiency in severe model ...... ntron retention and DNA damage
@en
SMN deficiency in severe model ...... tron retention and DNA damage.
@nl
prefLabel
SMN deficiency in severe model ...... ntron retention and DNA damage
@en
SMN deficiency in severe model ...... tron retention and DNA damage.
@nl
P2093
P2860
P356
P1476
SMN deficiency in severe model ...... ntron retention and DNA damage
@en
P2093
C Frank Bennett
Christina Fleet
Eric Chiao
Frank Rigo
Jessica A Hurt
John F Staropoli
John P Carulli
Mohini Jangi
Norm Allaire
Patrick Cullen
P2860
P304
E2347-E2356
P356
10.1073/PNAS.1613181114
P407
P577
2017-03-07T00:00:00Z