Duke University Duke University Medical Center Ophthalmology, Molecular Genetics and Microbiology Jules and Doris Stein Research to Prevent Blindness ProfessorAppointed: 2005 |  |
QualificationsPh.D., Purdue University, Neuroscience, 1993. B.Sc., Jacksonville State University, Biology, 1988. Licenciatura, University of Porto, Biological Sciences, 1988. Expertise and Research InterestsMy research program is broadly focused on the role of dynamic protein assembly complexes in intracellular transport, signaling and metabolism, organization of non- and -neuronal subcellular systems, and etiology and molecular pathogenesis of human diseases, with particular emphasis on neurodegenerative maladies causing blindness. Another area of focus is to determine the spatial and temporal dynamics between components of certain signaling multisubunit complexes on a molecular, cellular and physiological level, and developing proteomic, genetic and pharmacological strategies to probe such signaling machines. The neuroretina is the experimental model system of choice at this time for our studies because it is a well defined, diversified and stratified neuronal tissue, which is highly amenable to a wide array of manipulations in vivo and in vitro. The retinal neurons are superb tools to dissect biological processes ranging from a wide array of signal transduction mechanisms to protein biogenesis and kinesis and programmed cell death. Moreover, a vast number of inherited neuropathies lead to the death of selected neuronal cells in the human. Yet, the molecular pathogenesis of these neurodegenerative maladies is not understood. The research of my laboratory is currently centered specifically on dissecting i) the events underlying protein trafficking, signaling and biogenesis, specific metabolic steps and allied disease processes mediated by the vital and large scaffold and mosaic protein, Ran-binding protein 2 (RanBP2) and its molecular partners, and ii) the molecular pathogenesis of inherited neuroretinal disorders such as X-linked RP3, Leber congenital amaurosis, and allied congenital neuropathies. Other Expertise2002 - Present, Ad Hoc Reviewer, Integrative Biology and Neuroscience, National Science Foundation 2002 - Present, Ad Hoc Reviewer, The Wellcome Trust (U.K.) 2003 - Present, Ad Hoc Reviewer, Molecular and Cellular Biosiences, National Science Foundation 2006 - Present, Ad Hoc Reviewer, U.S.-Israel Binational Science Foundation KeywordsCOS Keywords:Biochemistry, Biological Sciences, Blindness, Cell Biology, Cell Physiology, Central Nervous System, Genetic Models, Genetics, Molecular Biology, Molecular Pathology, Neurobiology, Neurodegenerative Diseases, Neurology, Neuroscience, Pathobiology, Pharmacology, Proteins and Macromolecules, Retinitis Pigmentosa, Signal Transduction, Toxicology, Transgenic Animals, Vision.Additional Terms:Biochemistry, Blindness, Cell Biology, Central Nervous System, GTP-binding Proteins, GTPase Effectors, GTPase Modulators, Genetic Models, Genetics, Glucose Metabolism, Kinesins, Leber Congenital Amaurosis, Mitochondria, Molecular Biology, Molecular Pathology, Motor Proteins, Neurobiology, Neurodegenerative Diseases, Neuron, Nuclear Export, Nuclear Import, Nucleocytoplasmic Trafficking, Pathobiology, Pharmacology, Photoreceptor, Protein Biogenesis, Protein Kinesis, Protein Trafficking, RPGR, RPGRIP, Ran-Binding Proteins, RanBP2, RanGTPase, Retina, Retinitis Pigmentosa, Signal Transduction, Transgenic Animals, Vision.Languages(Reading, Writing, Speaking)Portuguese: (Fluent, Fluent, Fluent) Spanish: (Fluent, Basic, Functional) German: (Functional, Basic, Basic) MembershipsAmerican Society for Biochemistry and Molecular Biology American Society for Cell Biology Association for Research in Vision and Ophthalmology Biochemical Society Society for Experimental Biology and Medicine Society for Neuroscience Honors and Awards2007, Editorial Board,
Experimental Biology and Medicine
2007, Editorial Board,
The Open Ophthalmology Journal
2005-2009,
Jules and Doris Stein Research to Prevent Blindness Professorship Award,
Research to Prevent Blindness
1998-1999,
Research Award in Visual Sciences,
The Karl Kirchgessner Foundation
1993-1994,
Postdoctoral Science Fellowship,
N.A.T.O.
1993, A.H. Ismail Interdisciplinary Program Doctoral Research Award,
Purdue University
1991-1993,
SCIENCE Predoctoral Fellowship (E.C.),
E.C./F.C.T. (Portugal)
1989-1991,
Predoctoral Science Fellowship,
N.A.T.O.
Previous Positions2001-2005, Associate Professor,
Medical College of Wisconsin,
Pharmacology and Toxicology
1997-2001, Assistant Professor,
Medical College of Wisconsin,
Pharmacology and Toxicology
1994-1997, Research Fellow,
University of Texas Southwestern Medical Center at Dallas,
Neuroscience
1994-1997, Instructor,
University of Texas Southwestern Medical Center at Dallas,
Neuroscience
Funding Received- The Karl Kirchgessner Foundation:
,
Unrestricted,
Jun 1998
to Dec 1999.
- Research to Prevent Blindness:
Jules and Doris Stein Research to Prevent Blindness,
,
2005
to 2009.
- National Institutes of Health (NIH):
Molecular Pathogenesis of Retinitis Pigmentosa Type 3,
,
2001
to 2006.
- National Institutes of Health (NIH):
Structure-Function Analysis of RanBP2 in the Neuroretina,
,
1999
to 2007.
Publications- Cho KI, Cai Y, Yi H, Yeh A, Aslanukov A, Ferreira PA (2007) Association of the Kinesin-Binding Domain of RanBP2 to KIF5B and KIF5C Determines Mitochondria Localization and Function, Traffic, In Press
- Yi H, Friedman J, Ferreira PA (2007) The cyclophilin-like domain of Ran-binding protein-2 modulates selectively the activity of the ubiquitin-proteasome system and protein biogenesis, The Journal of Biological Chemistry, In Press
- Moore DF, Gelderman MP, Ferreira PA, Fuhrmann SR, Yi H, Elkahloun A, Lix LM, Brady RO, Schiffmann R, Goldin E (2007) Genomic abnormalities of the murine model of Fabry disease after disease-related perturbation, a systems biology approach, Proceedings of the National Academy of Sciences (USA), 104 (19), 8065-70
- Aslanukov, A., Bhowmick, R., Oswald, J., Lu, X., Raz, D., Sieving, P., Bock, C., Ferreira, P. (2006) Cox11 and Hexokinase I activities are modulated by RanBP2 and haploinsufficiency of RanBP2 causes deficits in glucose metabolism, PLoS Genetics, 2 (10), 1653-1665
- Roepman, R., Letteboer, S.,, Arts, H.H., C. van Beersum, S., Lu, X., Krieger, E., Ferreira, P.A., Cremers, F. M. (2005) Interaction of nephrocystin-4 and RPGRIP1 is disrupted by nephronophthisis or Leber congenital amaurosis (LCA)-associated mutations, Proceedings of the National Academy of Sciences (USA), 102, 18520-18525
- Ferreira, P. (2005) Insights Into X-linked RP3, Allied Diseases and Underlying Pathomechanisms, Human Molecular Genetics, 14 (2), R259-R267
- Lu X, Ferreira PA (Jun 2005) Identification of novel murine- and human-specific RPGRIP1 splice variants
with distinct expression profiles and subcellular localization., Investigative Ophthalmology & Visual Science, 46 (6), 1882-90
 - Lu X, Guruju M, Oswald J, Ferreira PA (May 2005) Limited proteolysis differentially modulates the stability and subcellular
localization of domains of RPGRIP1 that are distinctly affected by
mutations in Leber's congenital amaurosis., Human Molecular Genetics, 14 (10), 1327-40
 - Castagnet P, Mavlyutov T, Cai Y, Zhong F, Ferreira P, RPGRIP1s with distinct neuronal localization and biochemical properties
associate selectively with RanBP2 in amacrine neurons, Human Molecular Genetics, 12(15), 1847-63, August 2003
 - Mavlyutov TA, Cai Y, Ferreira PA, Identification of RanBP2- and kinesin-mediated transport pathways with
restricted neuronal and subcellular localization, Traffic (copenhagen, Denmark), 3(9), 630-40, September 2002
 - Mavlyutov TA, Zhao H, Ferreira PA, Species-specific subcellular localization of RPGR and RPGRIP isoforms:
implications for the phenotypic variability of congenital retinopathies
among species, Human Molecular Genetics, 11(16), 1899-907, Aug 2002
 - Cai Y, Singh BB, Aslanukov A, Zhao H, Ferreira PA, The docking of kinesins, KIF5B and KIF5C, to Ran-binding protein 2
(RanBP2) is mediated via a novel RanBP2 domain, Journal of Biological Chemistry, 276(45), 41594-602, November 2001
 - Fauser S, Aslanukov A, Roepman R, Ferreira PA, Genomic organization, expression, and localization of murine Ran-binding
protein 2 (RanBP2) gene, Mammalian Genome : Official Journal of the International Mammalian Genome Society, 12(6), 406-15, June 2001
 - Roepman R, Bernoud-Hubac N, Schick DE, Maugeri A, Berger W, Ropers HH, Cremers FP, Ferreira PA, The retinitis pigmentosa GTPase regulator (RPGR) interacts with novel
transport-like proteins in the outer segments of rod
photoreceptors, Human Molecular Genetics, 9(14), 2095-105, September 2000
 - Roepman R, Schick D, Ferreira PA, Isolation of retinal proteins that interact with retinitis pigmentosa
GTPase regulator by interaction trap screen in yeast, Methods in Enzymology, 316, 688-704, 2000
 - Ferreira PA, Characterization of RanBP2-associated molecular components in neuroretina, Methods in Enzymology, 315, 455-68, 2000
 - Singh BB, Patel HH, Roepman R, Schick D, Ferreira PA, The zinc finger cluster domain of RanBP2 is a specific docking site for the nuclear export factor, exportin-1, Journal of Biological Chemistry, 274(52), 37370-8, 1999
 - Ferreira PA, Yunfei C, Schick D, Roepman R, The cyclophilin-like domain mediates the association of Ran-binding protein 2 with subunits of the 19 S regulatory complex of the proteasome, Journal of Biological Chemistry, 273(38), 24676-82, 1998
 - Ferreira PA, Nakayama TA, Travis GH, Interconversion of red opsin isoforms by the cyclophilin-related chaperone protein Ran-binding protein 2, Proceedings of the National Academy of Sciences (USA), 94(4), 1556-61, 1997
 - Ferreira PA, Nakayama TA, Pak WL, Travis GH, Cyclophilin-related protein RanBP2 acts as chaperone for red/green opsin, Nature, 383(6601), 637-40, 1996
 - Ferreira PA, Hom JT, Pak WL, Retina-specifically expressed novel subtypes of bovine cyclophilin, Journal of Biological Chemistry, 270(39), 23179-88, 1995
 - Ferreira PA, Pak WL, Bovine phospholipase C highly homologous to the norpA protein of Drosophila is expressed specifically in cones, Journal of Biological Chemistry, 269(5), 3129-31, 1994
 - Ferreira PA, Shortridge RD, Pak WL, Distinctive subtypes of bovine phospholipase C that have preferential expression in the retina and high homology to the norpA gene product of Drosophila, Proceedings of the National Academy of Sciences (USA), 90(13), 6042-6, 1993

Profile DetailsCOS Expertise ID #1036234 Individual Expertise profile of Paulo A. Ferreira, Copyright Paulo A. Ferreira. © COS Expertise TM, 2008, ProQuest LLC All rights reserved. |