Gideon Dreyfuss

Gideon Dreyfuss
Born	New York City, New York, United States
Education	Hebrew University of Jerusalem (undergraduate), Harvard University (PhD), Massachusetts Institute of Technology (Post-doctoral)
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,
Scientific career
Fields	Biochemistry, Molecular Biology
Institutions	University of Pennsylvania, Howard Hughes Medical Institute, National Academy of Sciences, Northwestern University (formerly)
Thesis	On cyclic AMP-dependent protein kinases  (1978)
Doctoral advisor	Elkan Blout
Other academic advisors	David Baltimore

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

• eIF4A3^[2]
• FXR1^[3]
• FXR2^[4]
• Gemin2 (Previously known as SIP1)^[5]
• Gemin3/DDX20^[6]
• Gemin4^[7]
• Gemin5^[8]
• Gemin6^[9]
• Gemin7^[10]
• hnRNPA1^[11]
• RA33^[12]
• hnRNPC^[13]
• PTBP1^[14]
• hnRNPK^[15]
• hnRNPM^[16]
• hnRNPR^[17]
• hnRNPU^[18]
• hnRNPQ^[17]
• Y14^[19]
• Magoh^[20]

See also

• SMN1
• SMN2
• Spinal muscular atrophy
• Heterogeneous ribonucleoprotein particle
• Spliceosome

References

1. Penn Medicine Announcement, [1], May 3, 2012
2. Chan, C. C.; Dostie, J.; Diem, M. D.; Feng, W.; Mann, M.; Rappsilber, J.; Dreyfuss, G. (2004). "EIF4A3 is a novel component of the exon junction complex". RNA. 10 (2): 200–209. doi:10.1261/rna.5230104. PMC 1370532. PMID 14730019.
3. Siomi, M. C.; Siomi, H.; Sauer, W. H.; Srinivasan, S.; Nussbaum, R. L.; Dreyfuss, G. (1995). "FXR1, an autosomal homolog of the fragile X mental retardation gene". The EMBO Journal. 14 (11): 2401–2408. doi:10.1002/j.1460-2075.1995.tb07237.x. PMC 398353. PMID 7781595.
4. Zhang, Y.; O'Connor, J. P.; Siomi, M. C.; Srinivasan, S.; Dutra, A.; Nussbaum, R. L.; Dreyfuss, G. (1995). "The fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2". The EMBO Journal. 14 (21): 5358–5366. doi:10.1002/j.1460-2075.1995.tb00220.x. PMC 394645. PMID 7489725.
5. Liu, Q.; Fischer, U.; Wang, F.; Dreyfuss, G. (1997). "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. doi:10.1016/S0092-8674(00)80367-0. PMID 9323129. S2CID 10855404.
6. Charroux, B.; Pellizzoni, L.; Perkinson, R. A.; Shevchenko, A.; Mann, M.; Dreyfuss, G. (1999). "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. doi:10.1083/jcb.147.6.1181. PMC 2168095. PMID 10601333.
7. Charroux, B.; Pellizzoni, L.; Perkinson, R. A.; Yong, J.; Shevchenko, A.; Mann, M.; Dreyfuss, G. (2000). "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. doi:10.1083/jcb.148.6.1177. PMC 2174312. PMID 10725331.
8. Gubitz, A. K.; Mourelatos, Z.; Abel, L.; Rappsilber, J.; Mann, M.; Dreyfuss, G. (2001). "Gemin5, a Novel WD Repeat Protein Component of the SMN Complex That Binds Sm Proteins". Journal of Biological Chemistry. 277 (7): 5631–5636. doi:10.1074/jbc.M109448200. PMID 11714716.
9. Pellizzoni, L.; Baccon, J.; Rappsilber, J.; Mann, M.; Dreyfuss, G. (2001). "Purification of Native Survival of Motor Neurons Complexes and Identification of Gemin6 as a Novel Component". Journal of Biological Chemistry. 277 (9): 7540–7545. doi:10.1074/jbc.M110141200. PMID 11748230.
10. Baccon, J.; Pellizzoni, L.; Rappsilber, J.; Mann, M.; Dreyfuss, G. (2002). "Identification and Characterization of Gemin7, a Novel Component of the Survival of Motor Neuron Complex". Journal of Biological Chemistry. 277 (35): 31957–31962. doi:10.1074/jbc.M203478200. PMID 12065586.
11. Dreyfuss, G.; Choi, Y. D.; Adam, S. A. (1984). "Characterization of heterogeneous nuclear RNA-protein complexes in vivo with monoclonal antibodies". Molecular and Cellular Biology. 4 (6): 1104–1114. doi:10.1128/mcb.4.6.1104. PMC 368879. PMID 6204191.
12. Kamma, H.; Horiguchi, H.; Wan, L.; Matsui, M.; Fujiwara, M.; Fujimoto, M.; Yazawa, T.; Dreyfuss, G. (1999). "Molecular Characterization of the hnRNP A2/B1 Proteins: Tissue-Specific Expression and Novel Isoforms". Experimental Cell Research. 246 (2): 399–411. doi:10.1006/excr.1998.4323. PMID 9925756.
13. Choi, Y. D.; Dreyfuss, G. (1984). "Monoclonal antibody characterization of the C proteins of heterogeneous nuclear ribonucleoprotein complexes in vertebrate cells". The Journal of Cell Biology. 99 (6): 1997–2004. doi:10.1083/jcb.99.6.1997. PMC 2113551. PMID 6209285.
14. Ghetti, A.; Piñol-Roma, S.; Michael, W. M.; Morandi, C.; Dreyfuss, G. (1992). "HnRNP I, the polypyrimidine tract-binding protein: Distinct nuclear localization and association with hnRNAs". Nucleic Acids Research. 20 (14): 3671–3678. doi:10.1093/nar/20.14.3671. PMC 334017. PMID 1641332.
15. Siomi, H.; Matunis, M. J.; Michael, W. M.; Dreyfuss, G. (1993). "The pre-mRNA binding K protein contains a novel evolutionarily conserved motif". Nucleic Acids Research. 21 (5): 1193–1198. doi:10.1093/nar/21.5.1193. PMC 309281. PMID 8464704.
16. Datar, K. V.; Dreyfuss, G.; Swanson, M. S. (1993). "The human hnRNP M proteins: Identification of a methionine/arginine-rich repeat motif in ribonucleoproteins". Nucleic Acids Research. 21 (3): 439–446. doi:10.1093/nar/21.3.439. PMC 309137. PMID 8441656.
17. Mourelatos, Z.; Abel, L.; Yong, J.; Kataoka, N.; Dreyfuss, G. (2001). "SMN interacts with a novel family of hnRNP and spliceosomal proteins". The EMBO Journal. 20 (19): 5443–5452. doi:10.1093/emboj/20.19.5443. PMC 125643. PMID 11574476.
18. Kiledjian, M.; Dreyfuss, G. (1992). "Primary structure and binding activity of the hnRNP U protein: Binding RNA through RGG box". The EMBO Journal. 11 (7): 2655–2664. doi:10.1002/j.1460-2075.1992.tb05331.x. PMC 556741. PMID 1628625.
19. Kataoka, N.; Yong, J.; Kim, V. N.; Velazquez, F.; Perkinson, R. A.; Wang, F.; Dreyfuss, G. (2000). "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. doi:10.1016/S1097-2765(00)00065-4. PMID 11030346.
20. Kataoka, N.; Diem, M. D.; Kim, V. N.; Yong, J.; Dreyfuss, G. (2001). "Magoh, a human homolog of Drosophila mago nashi protein, is a component of the splicing-dependent exon-exon junction complex". The EMBO Journal. 20 (22): 6424–6433. doi:10.1093/emboj/20.22.6424. PMC 125744. PMID 11707413.

External links

• BMB: Gideon Dreyfuss
• Howard Hughes Medical Institute
• University of Pennsylvania CAMB: Gideon Dreyfuss
• Dreyfuss Lab Website