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10.1016/j.celrep.2017.01.059

http://scihub22266oqcxt.onion/10.1016/j.celrep.2017.01.059
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C5492976!5492976!28199839
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suck abstract from ncbi


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pmid28199839      Cell+Rep 2017 ; 18 (7): 1660-73
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  • The survival of motor neuron protein acts as a molecular chaperone for mRNP assembly #MMPMID28199839
  • Donlin-Asp PG; Fallini C; Campos J; Chou CC; Merritt ME; Phan HC; Bassell GJ; Rossoll W
  • Cell Rep 2017[Feb]; 18 (7): 1660-73 PMID28199839show ga
  • Spinal muscular atrophy (SMA) is a motor neuron disease caused by reduced levels of the survival of motor neuron (SMN) protein. SMN is part of a multiprotein complex that facilitates the assembly of spliceosomal small nuclear ribonucleoproteins (snRNPs). SMN has also been found to associate with mRNA binding proteins but the nature of this association was unknown. Here we have employed a combination of biochemical and advanced imaging methods to demonstrate that SMN promotes the molecular interaction between IMP1 protein and the 3? UTR zipcode region of ?-actin mRNA, leading to assembly of messenger ribonucleoprotein complexes (mRNPs) that associate with the cytoskeleton to facilitate trafficking. We have identified defects in mRNP assembly in cells and tissues from SMA disease models and patients that depend on the SMN Tudor domain and explain the observed deficiency in mRNA localization and local translation, providing insight into SMA pathogenesis as an RNP-assembly disorder.
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