Journalartikel

Jahr der Veröffentlichung: 2007

Seiten: 6608-6613

Zeitschrift: Proceedings of the National Academy of Sciences

Bandnummer: 104

Heftnummer: 16

ISSN: 0027-8424

eISSN: 1091-6490

Open Access Status: Green

DOI Link: https://doi.org/10.1073/pnas.0701919104

Verlag: National Academy of Sciences

Abstract: 
The spliceosome cycle consists of assembly, catalysis, and recycling phases. Recycling of postspliceosomal U4 and U6 small nuclear ribonucleoproteins (snRNPs) requires p110/SART3, a general splicing factor. In this article, we report that the zebrafish earl grey (egy) mutation maps in the p110 gene and results in a phenotype characterized by thymus hypoplasia, other organ-specific defects, and death by 7 to 8 days postfertilization. U4/U6 snRNPs were disrupted in egy mutant embryos, demonstrating the importance of p110 for U4/U6 snRNP recycling in vivo. Surprisingly, expression profiling of the egy mutant revealed an extensive network of coordinately up-regulated components of the spliceosome cycle, providing a mechanism compensating for the recycling defect. Together, our data demonstrate that a mutation in a general splicing factor can lead to distinct defects in organ development and cause disease.

Harvard-Zitierstil: Trede, N., Medenbach, J., Damianov, A., Hung, L., Weber, G., Paw, B., et al. (2007) Network of coregulated spliceosome components revealed by zebrafish mutant in recycling factor p110, Proceedings of the National Academy of Sciences, 104(16), pp. 6608-6613. https://doi.org/10.1073/pnas.0701919104

APA-Zitierstil: Trede, N., Medenbach, J., Damianov, A., Hung, L., Weber, G., Paw, B., Zhou, Y., Hersey, C., Zapata, A., Keefe, M., Barut, B., Stuart, A., Katz, T., Amemiya, C., Zon, L., & Bindereif, A. (2007). Network of coregulated spliceosome components revealed by zebrafish mutant in recycling factor p110. Proceedings of the National Academy of Sciences. 104(16), 6608-6613. https://doi.org/10.1073/pnas.0701919104