Research Topics
Genomes and Genes
| Livio PellizzoniSummaryAffiliation: Istituto di Biologia Cellulare Country: Italy Publications
| Collaborators
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Detail Information
Publications
Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel componentLivio Pellizzoni
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
J Biol Chem 277:7540-5. 2002..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...
Chaperoning ribonucleoprotein biogenesis in health and diseaseLivio Pellizzoni
Dulbecco Telethon Institute, Institute of Cell Biology CNR, Via E Ramarini 32, 00016 Monterotondo Scalo, Rome, Italy
EMBO Rep 8:340-5. 2007....- Gemin8 is required for the architecture and function of the survival motor neuron complexClaudia Carissimi
Dulbecco Telethon Institute, Institute of Cell Biology, Consiglio Nazionale delle Ricerche, Monterotondo Scalo, Rome 00016, Italy
J Biol Chem 281:37009-16. 2006.... - Gemin8 is a novel component of the survival motor neuron complex and functions in small nuclear ribonucleoprotein assemblyClaudia Carissimi
Dulbecco Telethon Institute, Institute of Cell Biology, Consiglio Nazionale delle Ricerche, 00016 Monterotondo Scalo, Rome, Italy
J Biol Chem 281:8126-34. 2006..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... - Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPsFrancesca Gabanella
Dulbecco Telethon Institute, Institute of Cell Biology, Monterotondo Scalo, Rome, Italy
PLoS ONE 2:e921. 2007..These findings are consistent with the hypothesis that SMN deficiency affects the splicing machinery and in particular the minor splicing pathway of a rare class of introns in SMA... - The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiationFrancesca Gabanella
Dulbecco Telethon Institute, Institute of Cell Biology CNR, Rome, Italy
Hum Mol Genet 14:3629-42. 2005..These results demonstrate that the SMN complex activity in snRNP assembly is regulated and point to a differential requirement for SMN function during development and cellular differentiation... - Unrip is a component of SMN complexes active in snRNP assemblyClaudia Carissimi
Dulbecco Telethon Institute, Institute of Cell Biology CNR, Monterotondo Scalo, Rome, Italy
FEBS Lett 579:2348-54. 2005..These results demonstrate that unrip functions in the pathway of snRNP biogenesis and is a marker of cellular SMN complexes active in snRNP assembly... - Essential role for the SMN complex in the specificity of snRNP assemblyLivio Pellizzoni
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
Science 298:1775-9. 2002..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... - The SMN complex is associated with snRNPs throughout their cytoplasmic assembly pathwaySéverine Massenet
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia 19104 6148, USA
Mol Cell Biol 22:6533-41. 2002..Thus, the SMN complex is associated with snRNPs during the entire process of their biogenesis in the cytoplasm and may have multiple functions throughout this process... - Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophyAmy M Avila
Neurogenetics Branch, National Institute of Neurological Disorders and Stroke NINDS, NIH, Bethesda, MA 20892, USA
J Clin Invest 117:659-71. 2007..These results indicate that the hydroxamic acid class of HDAC inhibitors activates SMN2 gene expression in vivo and has an ameliorating effect on the SMA disease phenotype when administered after disease onset... - Sequence-specific interaction of U1 snRNA with the SMN complexJeongsik Yong
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-6148, USA
EMBO J 21:1188-96. 2002..These findings indicate that SMN complex interaction with SL1 is sequence-specific and critical for U1 snRNP biogenesis, further supporting the direct role of the SMN complex in RNP biogenesis... - snRNAs contain specific SMN-binding domains that are essential for snRNP assemblyJeongsik Yong
Department of Biochemistry and Biophysics, Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104-6148, USA
Mol Cell Biol 24:2747-56. 2004..It is likely that this surveillance capacity of the SMN complex ensures assembly of Sm cores on the correct RNAs only and prevents illicit, potentially deleterious, assembly of Sm cores on random RNAs... - Identification and characterization of Gemin7, a novel component of the survival of motor neuron complexJennifer Baccon
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
J Biol Chem 277:31957-62. 2002..With the identification of Gemin7, the inventory of the core components of the SMN complex appears essentially complete...