http://www.cnr.it/ontology/cnr/individuo/prodotto/ID4175
Gemin8 Is Required for the Architecture and Function of the Survival Motor Neuron Complex*¡ (Articolo in rivista)
- Type
- Label
- Gemin8 Is Required for the Architecture and Function of the Survival Motor Neuron Complex*¡ (Articolo in rivista) (literal)
- Anno
- 2006-01-01T00:00:00+01:00 (literal)
- Alternative label
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
- Carissimi C., Saieva L., Gabanella F. and Pellizzoni L. (literal)
- Pagina inizio
- Pagina fine
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#descrizioneSinteticaDelProdotto
- Pubblicazione su rivista internazionale (literal)
- Note
- ISI Web of Science (WOS) (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
- Titolo
- Gemin8 Is Required for the Architecture and Function of the Survival Motor Neuron Complex*¡ (literal)
- Abstract
- The biogenesis of spliceosomal small nuclear ribonucleoproteins
(snRNPs) in higher eukaryotes requires the functions of
several cellular proteins and includes nuclear as well as cytoplasmic
phases. In the cytoplasm, a macromolecular complex containing
the survival motor neuron (SMN) protein, Gemin28
and Unrip mediates the ATP-dependent assembly of Sm proteins
and snRNAs into snRNPs. To carry out snRNP assembly,
the SMN complex binds directly to both Sm proteins and snRNAs;
however, the contribution of the individual components of
theSMNcomplex to its composition, interactions, and function
is poorly characterized. Here, we have investigated the functional
role of Gemin8 using novel monoclonal antibodies
against components of the SMN complex and RNA interference
experiments. We show that Gemin6, Gemin7, and Unrip form
a stable cytoplasmic complex whose association with SMN
requires Gemin8. Gemin8 binds directly to SMN and mediates
its interaction with the Gemin6/Gemin7 heterodimer. Importantly,
loss of Gemin6, Gemin7, and Unrip interaction with
SMN as a result of Gemin8 knockdown affects snRNP assembly
by impairing the SMN complex association with Sm proteins
but not with snRNAs. These results reveal the essential role of
Gemin8 for the proper structural organization of theSMNcomplex
and the involvement of the heteromeric subunit containing
Gemin6, Gemin7, Gemin8, and Unrip in the recruitment of Sm
proteins to the snRNP assembly pathway. (literal)
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