• Register
X
Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.

X

Leaving Community

Are you sure you want to leave this community? Leaving the community will revoke any permissions you have been granted in this community.

No
Yes

Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein.

Spinal muscular atrophy (SMA) is a genetic disorder caused by mutations in the human survival of motor neuron 1 gene, SMN1. SMN protein is part of a large complex that is required for biogenesis of various small nuclear ribonucleoproteins (snRNPs). Here, we report that SMN interacts directly with the Cajal body signature protein, coilin, and that this interaction mediates recruitment of the SMN complex to Cajal bodies. Mutation or deletion of specific RG dipeptide residues within coilin inhibits the interaction both in vivo and in vitro. Interestingly, GST-pulldown experiments show that coilin also binds directly to SmB'. Competition studies show that coilin competes with SmB' for binding sites on SMN. Ectopic expression of SMN and coilin constructs in mouse embryonic fibroblasts lacking endogenous coilin confirms that recruitment of SMN and splicing snRNPs to Cajal bodies depends on the coilin C-terminal RG motif. A cardinal feature of SMA patient cells is a defect in the targeting of SMN to nuclear foci; our results uncover a role for coilin in this process.

Pubmed ID: 11641277

Authors

  • Hebert MD
  • Szymczyk PW
  • Shpargel KB
  • Matera AG

Journal

Genes & development

Publication Data

October 15, 2001

Associated Grants

  • Agency: NIGMS NIH HHS, Id: GM53034
  • Agency: NINDS NIH HHS, Id: NS41617
  • Agency: NIGMS NIH HHS, Id: R01 GM053034
  • Agency: NINDS NIH HHS, Id: R01 NS041617
  • Agency: NICHD NIH HHS, Id: T32-HD07518

Mesh Terms

  • Amino Acid Sequence
  • Animals
  • Anura
  • Cell Line
  • Chickens
  • Coiled Bodies
  • Cyclic AMP Response Element-Binding Protein
  • DNA Primers
  • Fishes
  • Fluorescent Antibody Technique
  • Gene Expression
  • Genes, myc
  • Glutathione Transferase
  • HeLa Cells
  • Humans
  • Mice
  • Mice, Knockout
  • Molecular Sequence Data
  • Muscular Atrophy, Spinal
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • RNA-Binding Proteins
  • SMN Complex Proteins
  • Sequence Homology, Amino Acid
  • Survival of Motor Neuron 1 Protein
  • Transfection