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Direct interaction of Smn with dp103, a putative RNA helicase: A role for Smn in transcription regulation?
- Source :
- Scopus-Elsevier
-
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.
- Subjects :
- DNA, Complementary
Transcription, Genetic
animal diseases
Molecular Sequence Data
Nerve Tissue Proteins
Biology
DEAD-box RNA Helicases
Mice
Exon
DEAD Box Protein 20
SMN complex
Genes, Reporter
SMN Complex Proteins
Two-Hybrid System Techniques
Genetics
medicine
Transcriptional regulation
Animals
Humans
Tissue Distribution
Amino Acid Sequence
RNA, Messenger
Cyclic AMP Response Element-Binding Protein
Molecular Biology
Transcription factor
Genetics (clinical)
Sequence Homology, Amino Acid
Chromosome Mapping
RNA-Binding Proteins
Exons
General Medicine
Spinal muscular atrophy
Blotting, Northern
medicine.disease
Precipitin Tests
RNA Helicase A
Introns
nervous system diseases
Gene Expression Regulation
nervous system
Chromosomes, Human, Pair 1
RNA splicing
RNA Helicases
Protein Binding
Subjects
Details
- Database :
- OpenAIRE
- Journal :
- Scopus-Elsevier
- Accession number :
- edsair.doi.dedup.....9ad7fa05e3d873e179a1eac6fe537a7c