First Author | Pellizzoni L | Year | 2002 |
Journal | J Biol Chem | Volume | 277 |
Issue | 9 | Pages | 7540-5 |
PubMed ID | 11748230 | Mgi Jnum | J:75172 |
Mgi Id | MGI:2176021 | Doi | 10.1074/jbc.M110141200 |
Citation | Pellizzoni L, et al. (2002) Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component. J Biol Chem 277(9):7540-5 |
abstractText | The survival of motor neurons (SMN) protein, the product of the gene responsible for the motor neuron degenerative disease spinal muscular atrophy (SMA), is part of a large macromolecular complex. The SMN complex is localized in both the cytoplasm and the nucleus and contains SMN, Gemin2, Gemin3, Gemin4, Gemin5, and a few not yet identified proteins. The SMN complex plays a key role in the biogenesis of spliceosomal small nuclear ribonucleoproteins (snRNPs) and other ribonucleoprotein particles. As a step toward the complete characterization of the components of the SMN complex, we generated stable cell lines that express FLAG-tagged SMN or Gemin2 under the control of a tetracycline-inducible promoter. Native SMN complexes of identical protein composition to those isolated by immunoprecipitation with anti-SMN antibodies were purified by affinity chromatography from extracts of both cell lines. Here we report the identification by mass spectrometry of a novel protein component of the SMN complex termed Gemin6. Co-immunoprecipitation, immunolocalization, and in vitro binding experiments demonstrate that Gemin6 is a component of the SMN complex that localizes to gems and interacts with several Sm proteins of the spliceosomal snRNPs. |