Human Molecular Genetics, 2000, Vol. 9, No. 7 1093-1100
© 2000 Oxford University Press
Direct interaction of Smn with dp103, a putative RNA helicase: a role for Smn in transcription regulation?
Department of Human Anatomy and Genetics, and 2MRC Functional Genetics Unit, Department of Human Anatomy and Genetics, University of Oxford, South Parks Road, Oxford OX1 3QU, UK and 1Life Technologies Inc., 9800 Medical Center Drive, Rockville, MD 20849-6482, USA
Spinal muscular atrophy is an autosomal recessive neurodegenerative disease of childhood, resulting from deletion or mutation of the survival motor neuron (SMN) gene on chromosome 5q13. SMN exists as part of a 300 kDa multi-protein complex, incorporating several proteins critically required in pre-mRNA splicing. Although SMN mutations render SMN defective in this role, the specific 
-motor neuron degenerative phenotype seen in the disease remains unexplained. Here we demonstrate the isolation from mouse brain of the murine homologue of a recently identified novel RNA helicase of the DEAD box family, DP103, and its direct and specific binding of SMN. Previous work has shown that DP103 binds viral proteins known to interact with a cellular transcription factor to modulate gene expression. We suggest that the interaction between SMN and DP103 is further evidence for a role for SMN in transcriptional regulation and that SMN may be involved in the regulation of neuron-specific genes essential in neuronal development.
+ Present address: Mammalian Genetics Unit, MRC Harwell, Oxfordshire OX11 0RD, UK
§ To whom correspondence should be addressed. Tel: +44 1865 275317; Fax: +44 1865 272427; Email: kay.davies@anat.ox.ac.uk
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|
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