Abstract
The Scc2-Scc4 complex is essential for loading the cohesin complex onto DNA. Cohesin has important roles in chromosome segregation, DSB repair, and chromosome condensation. Here we report that Scc2 is important for gene expression in budding yeast. Scc2 and the transcriptional regulator Paf1 collaborate to promote the production of Box H/ACA snoRNAs which guide pseudouridylation of RNAs including ribosomal RNA. Mutation of SCC2 was associated with defects in the production of ribosomal RNA, ribosome assembly, and splicing. While the scc2 mutant does not have a general defect in protein synthesis, it shows increased frameshifting and reduced cap-independent translation. These findings suggest Scc2 normally promotes a gene expression program that supports translational fidelity. We hypothesize that translational dysfunction may contribute to the human disorder Cornelia de Lange syndrome, which is caused by mutations in NIPBL, the human ortholog of SCC2.
MeSH terms
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Cell Cycle Proteins / genetics
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Chromatin / genetics
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Chromosomal Proteins, Non-Histone / biosynthesis*
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Chromosomal Proteins, Non-Histone / genetics
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Cohesins
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De Lange Syndrome / genetics*
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De Lange Syndrome / pathology
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Gene Expression Regulation, Fungal
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Humans
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Protein Biosynthesis*
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Proteins / genetics*
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RNA Splicing / genetics
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RNA, Long Noncoding / biosynthesis*
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RNA, Long Noncoding / genetics
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RNA, Ribosomal / biosynthesis
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RNA, Ribosomal / genetics
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RNA, Small Nucleolar / biosynthesis
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RNA, Small Nucleolar / genetics
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Ribosomes / genetics
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Saccharomyces cerevisiae / genetics
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Saccharomyces cerevisiae Proteins / biosynthesis*
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Saccharomyces cerevisiae Proteins / genetics
Substances
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Cell Cycle Proteins
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Chromatin
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Chromosomal Proteins, Non-Histone
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NIPBL protein, human
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Proteins
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RNA, Long Noncoding
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RNA, Ribosomal
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RNA, Small Nucleolar
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SCC2 protein, S cerevisiae
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Saccharomyces cerevisiae Proteins
Grants and funding
The authors received no specific funding for this work.