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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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Sonraí Bibleagrafaíochta
Main Authors: Renvoisé, Benoît, Colasse, Sabrina, Burlet, Philippe, Viollet, Louis, Meier, U. Thomas, Lefebvre, Suzie
Formáid: Artigo
Teanga:Inglês
Foilsithe: Oxford University Press 2009
Ábhair:
Rochtain Ar Líne: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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