title: Direct interaction of Smn with dp103, a putative RNA helicase: a role for Smn in transcription regulation?
abstract: 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 alpha-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.
authors.affiliation: Department of Human Anatomy and Genetics, Oxford, UK.
authors.forename: L
authors.initials: L
authors.lastname: Campbell
authors.forename: K M
authors.initials: KM
authors.lastname: Hunter
authors.forename: P
authors.initials: P
authors.lastname: Mohaghegh
authors.forename: J M
authors.initials: JM
authors.lastname: Tinsley
authors.forename: M A
authors.initials: MA
authors.lastname: Brasch
authors.forename: K E
authors.initials: KE
authors.lastname: Davies
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D015152:Blotting, Northern; D002874:Chromosome Mapping; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics; D017362:Cyclic AMP Response Element-Binding Protein; D055541:DEAD Box Protein 20; D053487:DEAD-box RNA Helicases; D018076:DNA, Complementary / Q000378:metabolism; D005091:Exons; D005786:Gene Expression Regulation; D017930:Genes, Reporter; D006801:Humans; D007438:Introns; D051379:Mice; D008969:Molecular Sequence Data; D009419:Nerve Tissue Proteins / Q000378:metabolism* / Q000502:physiology*; D011233:Precipitin Tests; D011485:Protein Binding; D020365:RNA Helicases / Q000378:metabolism*; D012333:RNA, Messenger / Q000378:metabolism; D016601:RNA-Binding Proteins; D055532:SMN Complex Proteins; D017386:Sequence Homology, Amino Acid; D014018:Tissue Distribution; D014158:Transcription, Genetic; D020798:Two-Hybrid System Techniques
chemical_list: D017362:Cyclic AMP Response Element-Binding Protein; D018076:DNA, Complementary; D009419:Nerve Tissue Proteins; D012333:RNA, Messenger; D016601:RNA-Binding Proteins; D055532:SMN Complex Proteins; C120168:DDX20 protein, human; D055541:DEAD Box Protein 20; C485624:Ddx20 protein, mouse; D053487:DEAD-box RNA Helicases; D020365:RNA Helicases