DOI: 10.1126/science.1074962. Essential Role for the SMN Complex in the Specificity of snRNP Assembly [Penn]

Essential Role for the SMN Complex in the Specificity of snRNP Assembly

10.1126/science.1074962

Crossref journal-article

American Association for the Advancement of Science (AAAS)

Science (221)

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, nonspecific binding of Sm proteins to RNAs.

Bibliography

Pellizzoni, L., Yong, J., & Dreyfuss, G. (2002). Essential Role for the SMN Complex in the Specificity of snRNP Assembly. Science, 298(5599), 1775â1779.

Authors 3

Livio Pellizzoni (first)
Jeongsik Yong (additional)
Gideon Dreyfuss (additional)

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We thank members of our laboratory in particular A. Gubitz J. Baccon and S. Paushkin for stimulating discussions and for critical comments on this manuscript. We are grateful to J. Steitz for the anti-Sm Y12 monoclonal antibody. Supported by the Association Francaise Contre les Myopathies and by a grant from NIH. G.D. is an investigator of the Howard Hughes Medical Institute.

Dates

Type	When
Created	22 years, 8 months ago (Nov. 29, 2002, 10:32 a.m.)
Deposited	1 year, 7 months ago (Jan. 9, 2024, 9:02 p.m.)
Indexed	5 days, 7 hours ago (Aug. 21, 2025, 1:42 p.m.)
Issued	22 years, 8 months ago (Nov. 29, 2002)
Published	22 years, 8 months ago (Nov. 29, 2002)
Published Print	22 years, 8 months ago (Nov. 29, 2002)

Funders 0

None

BibTeX

@article{Pellizzoni_2002, title={Essential Role for the SMN Complex in the Specificity of snRNP Assembly}, volume={298}, ISSN={1095-9203}, url={http://dx.doi.org/10.1126/science.1074962}, DOI={10.1126/science.1074962}, number={5599}, journal={Science}, publisher={American Association for the Advancement of Science (AAAS)}, author={Pellizzoni, Livio and Yong, Jeongsik and Dreyfuss, Gideon}, year={2002}, month=nov, pages={1775–1779} }

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