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The loss of the snoRNP chaperone Nopp140 from Cajal bodies of patient fibroblasts correlates with the severity of spinal muscular atrophy

Spinal muscular atrophy (SMA) is a common autosomal recessive neurodegenerative disease caused by reduced survival motor neuron (SMN) levels. The assembly machinery containing SMN is implicated in the biogenesis of the spliceosomal small nuclear ribonucleoproteins (snRNPs). SMN is present in both th...

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Detalhes bibliográficos
Main Authors: Renvoisé, Benoît, Colasse, Sabrina, Burlet, Philippe, Viollet, Louis, Meier, U. Thomas, Lefebvre, Suzie
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2009
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2655770/
https://ncbi.nlm.nih.gov/pubmed/19129172
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddp009
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