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Papers In Press, published online ahead of print January 24, 2006
Dulbecco Telethon Institute, Monterotondo Scalo (Rome) 00016
Corresponding Author: livio.pellizzoni{at}ibc.cnr.it
The survival motor neuron (SMN) protein is the product of the spinal muscular atrophy (SMA) disease gene. SMN and Gemin2-7 proteins form a large macromolecular complex that localizes in the cytoplasm as well as in the nucleoplasm and in nuclear Gems. The SMN complex interacts with several additional proteins and likely functions in multiple cellular pathways. In the cytoplasm, a subset of SMN complexes containing unrip and Sm proteins mediate the assembly of spliceosomal small nuclear ribonucleoproteins (snRNPs). Here, by mass spectrometry analysis of SMN complexes purified from HeLa cells, we identify a novel protein that is evolutionarily conserved in metazoans and we name Gemin8. Co-immunoprecipitation and immunolocalization experiments demonstrate that Gemin8 is associated with the SMN complex and is localized in the cytoplasm and in the nucleus, where it is highly concentrated in Gems. Gemin8 interacts directly with the Gemin6-Gemin7 heterodimer and, together with unrip, these proteins form a heteromeric subunit of the SMN complex. Gemin8 is also associated with Sm proteins and Gemin8-containing SMN complexes are competent to carry out snRNP assembly. Importantly, RNA interference experiments indicate that Gemin8 knock-down impairs snRNP assembly and Gemin8 expression is down regulated in cells with low levels of SMN. These results demonstrate that Gemin8 is a novel integral component of the SMN complex and extend the repertoire of cellular proteins involved in the pathway of snRNP biogenesis.
J. Biol. Chem, 10.1074/jbc.M512243200
Submitted on November 14, 2005
Revised on December 21, 2005
Accepted on January 24, 2006
Gemin8 is a novel component of the survival motor neuron complex and functions in snRNP assembly
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