SMN Tudor domain structure and its interaction with the Sm proteins

@article{Selenko2001SMNTD,
  title={SMN Tudor domain structure and its interaction with the Sm proteins},
  author={Philipp Selenko and Remco Sprangers and Gunter Stier and Dirk Michael B{\"u}hler and Utz Fischer and Michael Sattler},
  journal={Nature Structural Biology},
  year={2001},
  volume={8},
  pages={27-31}
}
Spinal muscular atrophy (SMA) is a common motor neuron disease that results from mutations in the Survival of Motor Neuron (SMN) gene. The SMN protein plays a crucial role in the assembly of spliceosomal uridine-rich small nuclear ribonucleoprotein (U snRNP) complexes via binding to the spliceosomal Sm core proteins. SMN contains a central Tudor domain that facilitates the SMN–Sm protein interaction. A SMA-causing point mutation (E134K) within the SMN Tudor domain prevents Sm binding. Here, we… CONTINUE READING
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