Livio Pellizzoni

Summary

Affiliation: Istituto di Biologia Cellulare
Country: Italy

Publications

  1. ncbi request reprint Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component
    Livio 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
  2. pmc Chaperoning ribonucleoprotein biogenesis in health and disease
    Livio Pellizzoni
    Dulbecco Telethon Institute, Institute of Cell Biology CNR, Via E Ramarini 32, 00016 Monterotondo Scalo, Rome, Italy
    EMBO Rep 8:340-5. 2007
  3. ncbi request reprint Gemin8 is required for the architecture and function of the survival motor neuron complex
    Claudia Carissimi
    Dulbecco Telethon Institute, Institute of Cell Biology, Consiglio Nazionale delle Ricerche, Monterotondo Scalo, Rome 00016, Italy
    J Biol Chem 281:37009-16. 2006
  4. ncbi request reprint Gemin8 is a novel component of the survival motor neuron complex and functions in small nuclear ribonucleoprotein assembly
    Claudia Carissimi
    Dulbecco Telethon Institute, Institute of Cell Biology, Consiglio Nazionale delle Ricerche, 00016 Monterotondo Scalo, Rome, Italy
    J Biol Chem 281:8126-34. 2006
  5. pmc Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs
    Francesca Gabanella
    Dulbecco Telethon Institute, Institute of Cell Biology, Monterotondo Scalo, Rome, Italy
    PLoS ONE 2:e921. 2007
  6. ncbi request reprint The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation
    Francesca Gabanella
    Dulbecco Telethon Institute, Institute of Cell Biology CNR, Rome, Italy
    Hum Mol Genet 14:3629-42. 2005
  7. ncbi request reprint Unrip is a component of SMN complexes active in snRNP assembly
    Claudia Carissimi
    Dulbecco Telethon Institute, Institute of Cell Biology CNR, Monterotondo Scalo, Rome, Italy
    FEBS Lett 579:2348-54. 2005
  8. ncbi request reprint Essential role for the SMN complex in the specificity of snRNP assembly
    Livio 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
  9. pmc The SMN complex is associated with snRNPs throughout their cytoplasmic assembly pathway
    Séverine 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
  10. pmc Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy
    Amy M Avila
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke NINDS, NIH, Bethesda, MA 20892, USA
    J Clin Invest 117:659-71. 2007

Collaborators

  • Juri Rappsilber
  • Matthew E R Butchbach
  • C J Sumner
  • Claudia Carissimi
  • Francesca Gabanella
  • Jennifer Baccon
  • Jeongsik Yong
  • Gideon Dreyfuss
  • Luciano Saieva
  • Amy M Avila
  • Alan Sawyer
  • Pieranna Chiarella
  • Alessio Maiolica
  • Séverine Massenet
  • Nicholas A Di Prospero
  • Melanie A Knight
  • Barrington G Burnett
  • Kenneth H Fischbeck
  • Ziga Cizman
  • Parvana Hartenstein
  • Addis A Taye
  • Arthur H M Burghes
  • Alessandro Usiello
  • Marco Straccia
  • Daniel J Battle
  • Tracey J Golembe
  • Matthias Mann
  • Sergey Paushkin
  • Iain W Mattaj

Detail Information

Publications13

  1. ncbi request reprint Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component
    Livio 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...
  2. pmc Chaperoning ribonucleoprotein biogenesis in health and disease
    Livio Pellizzoni
    Dulbecco Telethon Institute, Institute of Cell Biology CNR, Via E Ramarini 32, 00016 Monterotondo Scalo, Rome, Italy
    EMBO Rep 8:340-5. 2007
    ....
  3. ncbi request reprint Gemin8 is required for the architecture and function of the survival motor neuron complex
    Claudia Carissimi
    Dulbecco Telethon Institute, Institute of Cell Biology, Consiglio Nazionale delle Ricerche, Monterotondo Scalo, Rome 00016, Italy
    J Biol Chem 281:37009-16. 2006
    ....
  4. ncbi request reprint Gemin8 is a novel component of the survival motor neuron complex and functions in small nuclear ribonucleoprotein assembly
    Claudia 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...
  5. pmc Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs
    Francesca 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...
  6. ncbi request reprint The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation
    Francesca 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...
  7. ncbi request reprint Unrip is a component of SMN complexes active in snRNP assembly
    Claudia 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...
  8. ncbi request reprint Essential role for the SMN complex in the specificity of snRNP assembly
    Livio 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...
  9. pmc The SMN complex is associated with snRNPs throughout their cytoplasmic assembly pathway
    Séverine 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...
  10. pmc Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy
    Amy 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...
  11. pmc Sequence-specific interaction of U1 snRNA with the SMN complex
    Jeongsik 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...
  12. pmc snRNAs contain specific SMN-binding domains that are essential for snRNP assembly
    Jeongsik 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...
  13. ncbi request reprint Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex
    Jennifer 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...