Essential role for the SMN complex in the specificity of snRNP assembly

Livio Pellizzoni, Jeongsik Yong, Gideon Dreyfuss

Research output: Contribution to journalArticlepeer-review

418 Scopus citations

Abstract

The Survival of Motor Neurons (SMN) protein, the product of the spinal muscular atrophy-determining gene, is part of a large macromolecular complex (SMN complex) that functions in the assembly of spliceosomal small nuclear ribonucleoproteins (snRNPs). Using cell extracts and purified components, we demonstrated that the SMN complex is necessary and sufficient to mediate the ATP-dependent assembly of the core of seven Sm proteins on uridine-rich, small nuclear ribonucleic acids (U snRNAs). In vitro experiments revealed strict requirements for ordered binding of the Sm proteins and the U snRNAs to the SMN complex. Importantly, the SMN complex is necessary to ensure that Sm cores assemble only on correct RNA targets and prevent their otherwise promiscuous association with other RNAs. Thus, the SMN complex functions as a specificity factor essential for the efficient assembly of Sm proteins on U snRNAs and likely protects cells from illicit, and potentially deleterious, non-specific binding of Sm proteins to RNAs.

Original languageEnglish (US)
Pages (from-to)1775-1779
Number of pages5
JournalScience
Volume298
Issue number5599
DOIs
StatePublished - Nov 29 2002

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