Research Topics
Genomes and Genes
| Gideon DreyfussSummaryAffiliation: University of Pennsylvania Country: USA Publications
Research Grants
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Detail Information
Publications
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...- A functional interaction between the survival motor neuron complex and RNA polymerase IIL Pellizzoni
Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
J Cell Biol 152:75-85. 2001..These findings indicate a functional as well as physical association of the SMN complex with pol II and suggest a role for the SMN complex in the assembly of the pol II transcription/processing machinery... - Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gemsB Charroux
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
J Cell Biol 147:1181-94. 1999..The presence of a DEAD box motif in Gemin3 suggests that it may provide the catalytic activity that plays a critical role in the function of the SMN complex on RNPs... - Gemin4. A novel component of the SMN complex that is found in both gems and nucleoliB Charroux
Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
J Cell Biol 148:1177-86. 2000..Interestingly, Gemin4 is also detected in the nucleoli, suggesting that the SMN complex may also function in preribosomal RNA processing or ribosome assembly... 
Messenger-RNA-binding proteins and the messages they carryGideon Dreyfuss
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
Nat Rev Mol Cell Biol 3:195-205. 2002..These proteins communicate crucial information to the translation machinery for the surveillance of nonsense mutations and for mRNA localization and translation...- Why do cells need an assembly machine for RNA-protein complexes?Jeongsik Yong
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
Trends Cell Biol 14:226-32. 2004..We discuss how this surveillance capacity of the SMN complex might ensure assembly of Sm cores only on the correct RNAs and prevent 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... - 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... - The Gemin6-Gemin7 heterodimer from the survival of motor neurons complex has an Sm protein-like structureYingli Ma
Department of Biochemistry and Biophysics, Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
Structure 13:883-92. 2005.... - miRNPs: a novel class of ribonucleoproteins containing numerous microRNAsZissimos Mourelatos
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
Genes Dev 16:720-8. 2002..The genomic sequences predict that miRNAs are likely to be derived from larger precursors that have the capacity to form stem-loop structures... - 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... - The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophyLili Wan
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
Mol Cell Biol 25:5543-51. 2005..Thus, SMN determines the capacity for snRNP biogenesis, and our findings provide evidence for a measurable deficiency in a biochemical activity in cells from patients with spinal muscular atrophy... - A novel WD repeat protein component of the methylosome binds Sm proteinsWestley J Friesen
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
J Biol Chem 277:8243-7. 2002.... - Lymphotropic Herpesvirus saimiri uses the SMN complex to assemble Sm cores on its small RNAsTracey J Golembe
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-6148, USA
Mol Cell Biol 25:602-11. 2005.... - 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... - SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicingZhenxi Zhang
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
Cell 133:585-600. 2008..These findings reveal a key role for the SMN complex in RNA metabolism and in splicing regulation and indicate that SMA is a general splicing disease that is not restricted to motor neurons... - The Gemin5 protein of the SMN complex identifies snRNAsDaniel J Battle
Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
Mol Cell 23:273-9. 2006..Gemin5 therefore functions as the factor that allows the SMN complex to distinguish snRNAs from other cellular RNAs for snRNP biogenesis... - Gemins modulate the expression and activity of the SMN complexWenqin Feng
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-6148, USA
Hum Mol Genet 14:1605-11. 2005.... - eIF4A3 is a novel component of the exon junction complexChia C Chan
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
RNA 10:200-9. 2004..Using monoclonal antibodies, we show that eIF4A3 is found in the nucleus whereas eIF4A1 and eIF4A2 are found in the cytoplasm. Thus, eIF4A3 likely provides a splicing-dependent influence on the translation of mRNAs... - The SMN complex, an assemblyosome of ribonucleoproteinsSergey Paushkin
Howard Hughes Medical Institute, and the Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
Curr Opin Cell Biol 14:305-12. 2002..This forms symmetrical dimethylarginines within the RG-rich domains of the substrates, thereby converting them to high-affinity binders of the SMN complex, and most likely providing regulation of the ribonucleoprotein assembly processes... - A quantitative high-throughput in vitro splicing assay identifies inhibitors of spliceosome catalysisMichael G Berg
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
Mol Cell Biol 32:1271-83. 2012..EJIPT provides a platform for characterization and discovery of splicing and EJC modulators... - PYM binds the cytoplasmic exon-junction complex and ribosomes to enhance translation of spliced mRNAsMichael D Diem
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
Nat Struct Mol Biol 14:1173-9. 2007..We suggest that PYM functions as a bridge between EJC-bearing spliced mRNAs and the translation machinery to enhance translation of the mRNAs... - SMN-independent subunits of the SMN complex. Identification of a small nuclear ribonucleoprotein assembly intermediateDaniel J Battle
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
J Biol Chem 282:27953-9. 2007..These findings demonstrate a modular nature of the SMN complex and identify a new intermediate in the snRNP assembly process... - Molecular functions of the SMN complexStephen J Kolb
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
J Child Neurol 22:990-4. 2007..This review focuses on the current understanding of the mechanism of the role of the SMN complex in small nuclear ribonucleoprotein assembly and considers the relationship of this function to spinal muscular atrophy... - Gemin5 delivers snRNA precursors to the SMN complex for snRNP biogenesisJeongsik Yong
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
Mol Cell 38:551-62. 2010..We propose a stepwise pathway of SMN complex formation and snRNP biogenesis, highlighting Gemin5's function in delivering pre-snRNAs as substrates for Sm core assembly and processing... - The SMN complexAmelie K Gubitz
Department of Biochemistry and Biophysics, Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-6148, USA
Exp Cell Res 296:51-6. 2004..A detailed understanding of the cellular roles of the SMN complex may help the development of therapeutic strategies for this neurodegenerative disease... - Specific sequence features, recognized by the SMN complex, identify snRNAs and determine their fate as snRNPsTracey J Golembe
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, 19104, USA
Mol Cell Biol 25:10989-1004. 2005..These findings demonstrate that the SMN complex is the identifier, as well as assembler, of the abundant class of snRNAs in cells because it is able to recognize an snRNP code that they contain... 
Gemin5-snRNA interaction reveals an RNA binding function for WD repeat domainsChi Kong Lau
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, USA
Nat Struct Mol Biol 16:486-91. 2009..The WD repeat domain is thus a previously undescribed RNA binding domain, and we suggest that the presence of WD repeats should be considered as predictive of potential function in RNA binding...- Ars2 promotes proper replication-dependent histone mRNA 3' end formationJoshua J Gruber
Abramson Family Cancer Research Institute, University of Pennsylvania, Philadelphia, PA 19104, USA
Mol Cell 45:87-98. 2012..Together, the data demonstrate that Ars2 contributes to histone mRNA 3' end formation and expression and these functional properties of Ars2 are negatively regulated by interaction with 7SK RNA... - Inactivation of the SMN complex by oxidative stressLili Wan
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
Mol Cell 31:244-54. 2008..Thus, the SMN complex is a redox-sensitive assemblyosome and an ROS target, suggesting that it may play a role in oxidative stress pathophysiology, which is associated with many degenerative diseases... - Numerous microRNPs in neuronal cells containing novel microRNAsJosee Dostie
Howard Hughes Medical Institute, and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
RNA 9:180-6. 2003..These subfamilies comprise multiple copies of miRNAs on different chromosomes, suggesting an important function in the regulation of gene expression... - A novel cell immunoassay to measure survival of motor neurons protein in blood cellsStephen J Kolb
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
BMC Neurol 6:6. 2006..A major goal of current therapeutic approaches is to increase SMN levels in SMA patients. The purpose of this study was to develop a reliable assay to measure SMN protein levels from peripheral blood samples... - 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... - tRNA binds to cytochrome c and inhibits caspase activationYide Mei
Department of Cancer Biology and Abramson Family Cancer Research Institute, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
Mol Cell 37:668-78. 2010..These findings suggest that tRNA, in addition to its well-established role in gene expression, may determine cellular responsiveness to apoptotic stimuli... - U1 snRNP protects pre-mRNAs from premature cleavage and polyadenylationDaisuke Kaida
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
Nature 468:664-8. 2010..These findings reveal a critical splicing-independent function for U1 snRNP in protecting the transcriptome, which we propose explains its overabundance... - Structure of a key intermediate of the SMN complex reveals Gemin2's crucial function in snRNP assemblyRundong Zhang
Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, USA
Cell 146:384-95. 2011..These findings provide insight into SMN complex assembly and specificity, linking snRNP biogenesis and SMA pathogenesis... - Rapid-response splicing reporter screens identify differential regulators of constitutive and alternative splicingIhab Younis
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
Mol Cell Biol 30:1718-28. 2010..Our findings reveal unexpected activities of clinically used drugs in splicing and uncover differential regulation of constitutively spliced introns... - Structure of the Y14-Magoh core of the exon junction complexChi Kong Lau
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6059, USA
Curr Biol 13:933-41. 2003..This tenacious, persistent, splicing-dependent, yet RNA sequence-independent, association suggests an important signaling function and must require distinct structural features for these proteins... - Translation is required to remove Y14 from mRNAs in the cytoplasmJosee Dostie
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
Curr Biol 12:1060-7. 2002..How Y14 and other components of the EJC are removed from mRNAs into the cytoplasm has not been determined... - Ars2 links the nuclear cap-binding complex to RNA interference and cell proliferationJoshua J Gruber
Abramson Family Cancer Research Institute and Department of Cancer Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
Cell 138:328-39. 2009..These findings provide evidence for a role for Ars2 in RNA interference regulation during cell proliferation... - A degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severitySungchan Cho
Howard Hughes Medical Institute and Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
Genes Dev 24:438-42. 2010..These findings explain a key aspect of the SMA disease mechanism, and suggest new treatment approaches based on interference with SMNDelta7-DEG activity... - RNA-binding proteins and post-transcriptional gene regulationTina Glisovic
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6148, United States
FEBS Lett 582:1977-86. 2008..In this short review, we focus on the RBPs that interact with pre-mRNAs and mRNAs and discuss their roles in the regulation of post-transcriptional gene expression... - Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteinsAmelie K Gubitz
Howard Hughes Medical Institute, Department of Biochemistry, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6148, USA
J Biol Chem 277:5631-6. 2002..The presence of 13 WD repeat domains in the amino-terminal half of Gemin5 and a coiled-coil motif near its carboxyl terminus indicate that it may form a large heteromeric complex and engage in multiple interactions... - Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degenerationManuela Neumann
Center for Neuropathology and Prion Research, Ludwig Maximilians University, Feodor Lynen Str 23, 81377 Munich, Germany
Acta Neuropathol 113:543-8. 2007..These results argue against a role of these binding partners in the pathogenesis of FTLD-U and emphasize the specificity of TDP-43 as marker for FTLD-U pathology... - Binding of a novel SMG-1-Upf1-eRF1-eRF3 complex (SURF) to the exon junction complex triggers Upf1 phosphorylation and nonsense-mediated mRNA decayIsao Kashima
Department of Molecular Biology, Yokohama City University School of Medicine, Yokohama 236 0004, Japan
Genes Dev 20:355-67. 2006..Thus, the SMG-1-mediated phosphorylation of Upf1 occurs on the association of SURF with EJC, which provides the link between the EJC and recognition of PTCs and triggers NMD... - A simple whole cell lysate system for in vitro splicing reveals a stepwise assembly of the exon-exon junction complexNaoyuki Kataoka
Institute for Virus Research, Kyoto University, Kyoto 606, Japan
J Biol Chem 279:7009-13. 2004..These findings indicate that EJC formation is an ordered pathway that involves stepwise association of components and is coupled to specific intermediates of the splicing reaction... - Where in the cell is the minor spliceosome?Joan A Steitz
Yale University and Howard Hughes Medical Institute, New Haven, CT 06536, USA
Proc Natl Acad Sci U S A 105:8485-6. 2008 - Ce-Y14 and MAG-1, components of the exon-exon junction complex, are required for embryogenesis and germline sexual switching in Caenorhabditis elegansTaizo Kawano
Department of Life Science, Graduate School of Science and Technology, Kobe University, 1 1 Rokkodaicho, Nadaku, Kobe 657 8501, Japan
Mech Dev 121:27-35. 2004..Our results demonstrate that the evolutionarily conserved interaction between two EJC components, Ce-Y14 and MAG-1, has critical developmental roles in C. elegans...
Research Grants
- FUNCTIONS OF SMN - THE SPINAL MUSCULAR ATROPHY PROTEINGideon Dreyfuss; Fiscal Year: 2002..Studies will be carried out to understand in details the molecular functions and interactions of the SMN protein and of the SMN complex. From these studies approaches to therapy of SMA are likely to emerge. ..