Gideon Dreyfuss Explained

Gideon Dreyfuss
Birth Place:New York City, New York, United States
Nationality:American
Field:Biochemistry, Molecular Biology
Work Institution:University of Pennsylvania,
Howard Hughes Medical Institute,
National Academy of Sciences,
Northwestern University (formerly)
Alma Mater:Hebrew University of Jerusalem (undergraduate),
Harvard University (PhD),
Massachusetts Institute of Technology (Post-doctoral)
Academic Advisors:David Baltimore
Doctoral Advisor:Elkan Blout
Thesis Title:On cyclic AMP-dependent protein kinases
Thesis Year:1978
Known For:Discovering and naming several hnRNP's and the proteins associated with SMN, for which loss of function is the primary cause of SMA,

Gideon Dreyfuss is an American biochemist, the Isaac Norris Professor of Biochemistry and Biophysics at the University of Pennsylvania School of Medicine, and an investigator of the Howard Hughes Medical Institute. He was elected to the National Academy of Sciences in 2012.[1]

Dreyfuss received his Ph.D. in biological chemistry in 1978 from Harvard University and is a fellow of the American Academy of Arts and Sciences.

Research

The Dreyfuss Lab is interested in various projects studying the function and biogenesis of non-coding RNA and the proteins that interact with RNA. A primary research goal of the lab is to elucidate the function of Survival of Motor Neuron protein, SMN, which assembles a heptameric ring of Sm proteins on U snRNAs to form snRNPs that are essential components of the splicesome. Moreover, loss of functional SMN is directly linked to spinal muscular atrophy, a debilitating neurodegenerative disease that is characterize by the eventual death of motor neurons and muscular wasting. The Dreyfuss Lab is conducting research to understand the role of SMN in SMA pathology and using high throughput screening to discover potential therapeutics. The lab also studies the dynamic mechanism of RNA splicing, the RNA-binding proteins that determine exonic specificity, and snRNAs, that are important regulators of splicing and mRNA maturation.

Genes or Gene Functions Discovered

See also

External links

Notes and References

  1. Penn Medicine Announcement, http://www.uphs.upenn.edu/news/News_Releases/2012/05/three/, May 3, 2012
  2. Chan . C. C. . Dostie . J. . Diem . M. D. . Feng . W. . Mann . M. . Rappsilber . J. . Juri Rappsilber. Dreyfuss . G. . EIF4A3 is a novel component of the exon junction complex . RNA . 10 . 2 . 200–209 . 2004 . 14730019 . 1370532 . 10.1261/rna.5230104.
  3. Siomi . M. C. . Siomi . H. . Sauer . W. H. . Srinivasan . S. . Nussbaum . R. L. . Dreyfuss . G. . FXR1, an autosomal homolog of the fragile X mental retardation gene . The EMBO Journal . 14 . 11 . 2401–2408 . 1995 . 7781595 . 398353 . 10.1002/j.1460-2075.1995.tb07237.x.
  4. Zhang . Y. . O'Connor . J. P. . Siomi . M. C. . Srinivasan . S. . Dutra . A. . Nussbaum . R. L. . Dreyfuss . G. . The fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2 . The EMBO Journal . 14 . 21 . 5358–5366 . 1995 . 7489725 . 394645 . 10.1002/j.1460-2075.1995.tb00220.x.
  5. 10.1016/S0092-8674(00)80367-0 . Liu . Q. . Fischer . U. . Wang . F. . Dreyfuss . G. . The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins . Cell . 90 . 6 . 1013–1021 . 1997 . 9323129. 10855404 . free .
  6. 10.1083/jcb.147.6.1181 . Charroux . B. . Pellizzoni . L. . Perkinson . R. A. . Shevchenko . A. . Mann . M. . Dreyfuss . G. . Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems . The Journal of Cell Biology . 147 . 6 . 1181–1194 . 1999 . 10601333 . 2168095.
  7. 10.1083/jcb.148.6.1177 . Charroux . B. . Pellizzoni . L. . Perkinson . R. A. . Yong . J. . Shevchenko . A. . Mann . M. . Dreyfuss . G. . Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli . The Journal of Cell Biology . 148 . 6 . 1177–1186 . 2000 . 10725331 . 2174312.
  8. Gubitz . A. K. . Mourelatos . Z. . Abel . L. . Rappsilber . J. . Mann . M. . Dreyfuss . G. . Gemin5, a Novel WD Repeat Protein Component of the SMN Complex That Binds Sm Proteins . 10.1074/jbc.M109448200 . Journal of Biological Chemistry . 277 . 7 . 5631–5636 . 2001 . 11714716 . free .
  9. Pellizzoni . L. . Baccon . J. . Rappsilber . J. . Mann . M. . Dreyfuss . G. . Purification of Native Survival of Motor Neurons Complexes and Identification of Gemin6 as a Novel Component . 10.1074/jbc.M110141200 . Journal of Biological Chemistry . 277 . 9 . 7540–7545 . 2001 . 11748230 . free .
  10. Baccon . J. . Pellizzoni . L. . Rappsilber . J. . Mann . M. . Dreyfuss . G. . Identification and Characterization of Gemin7, a Novel Component of the Survival of Motor Neuron Complex . 10.1074/jbc.M203478200 . Journal of Biological Chemistry . 277 . 35 . 31957–31962 . 2002 . 12065586 . free .
  11. Dreyfuss . G. . Choi . Y. D. . Adam . S. A. . Characterization of heterogeneous nuclear RNA-protein complexes in vivo with monoclonal antibodies . Molecular and Cellular Biology . 4 . 6 . 1104–1114 . 1984 . 6204191 . 368879 . 10.1128/mcb.4.6.1104.
  12. Kamma . H. . Horiguchi . H. . Wan . L. . Matsui . M. . Fujiwara . M. . Fujimoto . M. . Yazawa . T. . Dreyfuss . G. . 10.1006/excr.1998.4323 . Molecular Characterization of the hnRNP A2/B1 Proteins: Tissue-Specific Expression and Novel Isoforms . Experimental Cell Research . 246 . 2 . 399–411 . 1999 . 9925756 .
  13. Choi . Y. D. . Dreyfuss . G. . Monoclonal antibody characterization of the C proteins of heterogeneous nuclear ribonucleoprotein complexes in vertebrate cells . The Journal of Cell Biology . 99 . 6 . 1997–2004. 1984 . 6209285 . 2113551 . 10.1083/jcb.99.6.1997.
  14. 10.1093/nar/20.14.3671 . Ghetti . A. . Piñol-Roma . S. . Michael . W. M. . Morandi . C. . Dreyfuss . G. . HnRNP I, the polypyrimidine tract-binding protein: Distinct nuclear localization and association with hnRNAs . Nucleic Acids Research . 20 . 14 . 3671–3678 . 1992 . 1641332 . 334017.
  15. Siomi . H. . Matunis . M. J. . Michael . W. M. . Dreyfuss . G. . The pre-mRNA binding K protein contains a novel evolutionarily conserved motif . Nucleic Acids Research . 21 . 5 . 1193–1198 . 1993 . 8464704 . 309281 . 10.1093/nar/21.5.1193.
  16. Datar . K. V. . Dreyfuss . G. . Swanson . M. S. . The human hnRNP M proteins: Identification of a methionine/arginine-rich repeat motif in ribonucleoproteins . Nucleic Acids Research . 21 . 3 . 439–446 . 1993 . 8441656 . 309137 . 10.1093/nar/21.3.439.
  17. Mourelatos . Z. . Abel . L. . Yong . J. . Kataoka . N. . Dreyfuss . G. . SMN interacts with a novel family of hnRNP and spliceosomal proteins . 10.1093/emboj/20.19.5443 . The EMBO Journal . 20 . 19 . 5443–5452 . 2001 . 11574476 . 125643 .
  18. Kiledjian . M. . Dreyfuss . G. . Primary structure and binding activity of the hnRNP U protein: Binding RNA through RGG box . The EMBO Journal . 11 . 7 . 2655–2664 . 1992 . 1628625 . 556741 . 10.1002/j.1460-2075.1992.tb05331.x.
  19. 10.1016/S1097-2765(00)00065-4 . Kataoka . N. . Yong . J. . Kim . V. N. . Velazquez . F. . Perkinson . R. A. . Wang . F. . Dreyfuss . G. . Pre-mRNA splicing imprints mRNA in the nucleus with a novel RNA-binding protein that persists in the cytoplasm . Molecular Cell . 6 . 3 . 673–682 . 2000 . 11030346. free .
  20. Kataoka . N. . Diem . M. D. . Kim . V. N. . Yong . J. . Dreyfuss . G. . Magoh, a human homolog of Drosophila mago nashi protein, is a component of the splicing-dependent exon-exon junction complex . 10.1093/emboj/20.22.6424 . The EMBO Journal . 20 . 22 . 6424–6433 . 2001 . 11707413 . 125744 .

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