http://www.cnr.it/ontology/cnr/individuo/prodotto/ID4173
Gemin8 Is a Novel Component of the Survival Motor Neuron Complex and Functions in Small Nuclear Ribonucleoprotein Assembly* (Articolo in rivista)
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- Label
- Gemin8 Is a Novel Component of the Survival Motor Neuron Complex and Functions in Small Nuclear Ribonucleoprotein Assembly* (Articolo in rivista) (literal)
- Anno
- 2006-01-01T00:00:00+01:00 (literal)
- Alternative label
Carissimi C., Saieva L., Baccon J., Chiarella P., Maiolica A., Sawyer A., Rappsilber J. and Pellizzoni L. (2006)
Gemin8 Is a Novel Component of the Survival Motor Neuron Complex and Functions in Small Nuclear Ribonucleoprotein Assembly*
(literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
- Carissimi C., Saieva L., Baccon J., Chiarella P., Maiolica A., Sawyer A., Rappsilber J. and Pellizzoni L. (literal)
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- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#descrizioneSinteticaDelProdotto
- Pubblicazione su rivista internazionale (literal)
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- ISI Web of Science (WOS) (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
- Titolo
- Gemin8 Is a Novel Component of the Survival Motor Neuron Complex and Functions in Small Nuclear Ribonucleoprotein Assembly* (literal)
- Abstract
- The survival motor neuron (SMN) protein is the product of the spinal
muscular atrophy disease gene.SMNand Gemin27 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 mediates the assembly of spliceosomal
small nuclear ribonucleoproteins (snRNPs). Here, by mass spectrometry
analysis ofSMNcomplexes purified from HeLa cells, we identified
a novel protein that is evolutionarily conserved in metazoans, and we
named it Gemin8. Co-immunoprecipitation and immunolocalization
experiments demonstrated that Gemin8 is associated with the SMN
complexandislocalizedinthecytoplasmandinthenucleus,whereitis
highly concentrated in Gems. Gemin8 interacts directly with the
Gemin6-Gemin7 heterodimer and, together with unrip, these proteins
form a heteromeric subunit of theSMNcomplex. 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. (literal)
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