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Human Molecular Genetics Advance Access published online on July 17, 2007
Human Molecular Genetics, doi:10.1093/hmg/ddm192
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© The Author 2007. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Targeting of SMN to Cajal bodies is mediated by self-association

Robert Morse, Debra J. Shaw, Adrian G. Todd and Philip J. Young*

Clinical Neurobiology, Institute of Biomedical and Clinical Science, Peninsula College of Medicine and Dentistry, Exeter, EX1 2LU

* To whom correspondence should be addressed: Clinical Neurobiology, Institute of Biomedical and Clinical, Science, Peninsula College of Medicine and Dentistry, St. Luke's Campus, Exeter, EX1 2LU, Tel: 01392 26 29 39, Fax: 01392 26 29 26, E-mail: philip.young{at}pms.ac.uk

Received May 17, 2007; Revised July 14, 2007; Accepted July 14, 2007

The childhood autosomal recessive disorder Spinal Muscular Atrophy (SMA) is caused by mutations in the Survival Motor Neuron (SMN) gene. SMN localises diffusely in the cytoplasm and in distinct nuclear structures called Cajal bodies. Cajal bodies are believed to be storage and processing sites of several ribonucleoproteins (RNPs). Here, using a novel panel of SMN exon deletion constructs, we report a systematic analysis of internal targeting domains in the SMN protein. We demonstrate that the peptides encoded by exons 2b, 3 and 6 perform an integral role in the cellular targeting of SMN. In addition, we identify a nine amino acid motif within the highly conserved sequences of the exon 2b encoded-domain that mediates Cajal body targeting and self-association. Deletion of this domain dramatically affects SMN activity and results in a dominant-negative clone. These results identify critical domains within the SMN protein and have an impact on our understanding of the SMN protein with regards to SMA as well as cellular biology.


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