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  Protein 61K, encoded by a gene (PRPF31) linked to autosomal dominant retinitis pigmentosa, is required for U4/U6 center dot U5 tri-snRNP formation and pre-mRNA splicing

Makarova, O., Makarov, E. M., Liu, S., Vornlocher, H. P., & Luehrmann, R. (2002). Protein 61K, encoded by a gene (PRPF31) linked to autosomal dominant retinitis pigmentosa, is required for U4/U6 center dot U5 tri-snRNP formation and pre-mRNA splicing. EMBO Journal, 21(5), 1148-1157. Retrieved from http://www.nature.com/emboj/journal/v21/n5/pdf/7594333a.pdf.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-0012-F419-1 Version Permalink: http://hdl.handle.net/11858/00-001M-0000-002A-0938-A
Genre: Journal Article

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 Creators:
Makarova, O.1, Author              
Makarov, E. M.1, Author              
Liu, S.2, Author              
Vornlocher, H. P., Author
Luehrmann, R.1, Author              
Affiliations:
1Department of Cellular Biochemistry, MPI for biophysical chemistry, Max Planck Society, ou_578576              
2Research Group of X-Ray Crystallography, MPI for biophysical chemistry, Max Planck Society, ou_578578              

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Free keywords: pre-mRNA splicing; retinitis pigmentosa; snRNP protein function; spliceosome assembly; U4/U6 center dot U5 tri-snRNPs
 Abstract: In each round of nuclear pre-mRNA splicing, the U4/ U6.U5 tri- snRNP must be assembled from U4/U6 and U5 snRNPs, a reaction that is at present poorly understood. We have characterized a 61 kDa protein (61K) found in human U4/U6.U5 tri-snRNPs, which is homologous to yeast Prp31p, and show that it is required for this step. Immunodepletion of protein 61K from HeLa nuclear extracts inhibits tri-snRNP formation and subsequent spliceosome assembly and pre-mRNA splicing. Significantly, complementation with recombinant 61K protein restores each of these steps. Protein 61K is operationally defined as U4/U6 snRNP-specific as it remains bound to this particle at salt concentrations where the tri-snRNP dissociates. However, as shown by two-hybrid analysis and biochemical assays, protein 61K also interacts specifically with the U5 snRNP-associated 102K protein, indicating that it physically tethers U4/U6 to the U5 snRNP to yield the tris-nRNP. Interestingly, protein 61K is encoded by a gene (PRPF31) that has been shown to be linked to autosomal dominant retinitis pigmentosa. Thus, our studies suggest that disruptions in tri-snRNP formation and function resulting from mutations in the 61K protein may contribute to the manifestation of this disease.

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Language(s): eng - English
 Dates: 2002-03-01
 Publication Status: Published in print
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 Rev. Method: Peer
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Title: EMBO Journal
Source Genre: Journal
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Pages: - Volume / Issue: 21 (5) Sequence Number: - Start / End Page: 1148 - 1157 Identifier: -