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Proc Natl Acad Sci U S A. 2007 Apr 17;104(16):6608-13. Epub 2007 Apr 6.

Network of coregulated spliceosome components revealed by zebrafish mutant in recycling factor p110.

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  • 1Institute of Biochemistry, Justus-Liebig-University, D-35392 Giessen, Germany.

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.

PMID:
17416673
[PubMed - indexed for MEDLINE]
PMCID:
PMC1871833
Free PMC Article
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