Name Federico Gonzalez-Fernandez
Credentials MD, PhD (neuroscience)
Clinical Specialty Ocular Pathology
Title Ira Gile Ross & Elizabeth Pierce Olmsted Ross, MD Endowed Chair Professor of Ophthalmology
Office Address (research laboratory):
Medical Research Service
Veterans Affairs Medical Center
Building 20, Room 219
3495 Bailey Avenue
Buffalo, New York.
Phone (716) 863-2291
Email fg23@buffalo.edu
Education and Training:
Medical and Graduate School
Medical Scientist Training Program, Baylor College of Medicine, Houston, TX
Residency (Pathology)
University of Virginia School of Medicine, Charlottesville, VA
Certification: Anatomic Pathology (American Board of Pathology).
Fellowships
Cullen Eye Institute, Baylor College of Medicine (Molecular Biology)
University of Virginia School of Medicine, Department of Pathology (Neuropathology)
Research Interest
The vertebrate eye forms through the invagination of the optic vesicle allowing the neural retina, and retinal pigmented epithelium (RPE)/choroid to come into direct apposition in a bi-layered “optic cup”. This sets the stage for a variety of fascinating collaborations between the two layers in the embryonic, and adult retina ranging from developmental interactions (e.g. cellular induction), physiological processes (e.g. vitamin A cycle, retinal adhesion), and pathological states (including degenerations and retinal detachment).
Our laboratory is broadly interested in the retina-RPE/choroid as a model of cell-cell interactions mediated through extracellular matrices. In fact, there are few places in the body as well suited to study such interactions. Consider that the interphotoreceptor matrix (IPM), which fills the subretinal space, is sandwiched between the neural retina and apical RPE surface. In turn, the basal RPE contacts Bruch’s membrane (a complex matrix formed by the basement membranes of the RPE and choroidal capillaries). Unlike other CNS matrices, the IPM and Bruch’s membrane can be readily isolated.
The major soluble component of the IPM is a 145 kDa glycolipoprotein termed interphotoreceptor retinoid-binding protein (IRBP). IRBP, which is secreted by the rods and cones, is sequestered in the IPM, and has been thought to play a role in retina–RPE/choroid interactions. Although its role is far from understood, an emerging picture is that at least one function of IRBP is to transport, target, and protect 11-cis retinol, all-trans retinol, and 11-cis retinal trafficking between the photoreceptors, RPE and Müller cells in the vitamin A cycle. Understanding how IRBP accomplishes these remarkable tasks is a focus of our group. Our experimental approaches, which embrace Xenopus transgenic technology, zebrafish biology, molecular and biochemical methods, to X-ray crystallography, provide a rich environment for pre- and postdoctoral projects. Finally, as director of the Ophthalmic Pathology Service I bring a perspective of the pathophysiology of ocular disease, broadening the trainee’s appreciation of vision research.
Current Laboratory Staff and Students
Molly Sprada (Laboratory Technician, VA Research lab)
Savannah Davis (Laboratory Assistant, Ross Pathology Lab)
Cheryl Dann (Animal Technician)
Veronica Gonzalez-Fernandez (Office Assistant)
Dr. Dongjin Sung “Luke” (Postdoctoral Research Scholar)
Mary Alice Garlipp MS (PhD candidate, Neuroscience)
Tony Sacca (Graduate Research Assistant, Neuroscience)
Undergraduate Research Assistants: Priscilla Gonzalez-Fernandez, Alex Sunshine, Alex McCrossen, and Christina Garcia
Research Funding
NEI RO1 EY 09412-12
Veterans Affairs Office of Research and Development Award I01BX007080
Core Infrastructure Grant R24 EY 016662
Unrestricted departmental grant, Research to Prevent Blindness
Knights Templar Eye Foundation
Grant in Aid, Research to Prevent Blindness
National Society to Prevent Blindness
American Cancer Society
Selected Publications
1. Gonzalez-Fernandez, F. (2010). Development of the Retina. In Pediatric Retina, J.D. Reynolds, Olitsky, S.E., ed. (New York, Springer), pp. 1-38
2. Gonzalez-Fernandez F, Bevilacqua T, Lee KI, Chandrashekar R, Hsu L, Garlipp MA, Griswold JB, Crouch RK, Ghosh D. 2009. Retinol-binding site in interphotoreceptor retinoid-binding protein (IRBP): a novel hydrophobic cavity. Invest Ophthal Vis Sci 50:5577-86.
3. den Hollander AI, McGee TL, Ziviello C, Banfi S, Dryja TP, Gonzalez-Fernandez F, Ghosh D, Berson EL. 2009. A homozygous missense mutation in the IRBP gene (RBP3) associated with autosomal recessive retinitis pigmentosa. Invest Ophthalmol Vis Sci 50: 1864-72
4. Gonzalez-Fernandez, F., Ghosh, D. (2008). Focus on molecules: Interphotoreceptor retinoid-binding protein (IRBP). Exp. Eye Res. 86(2):169-170.
5. Gonzalez-Fernandez, F., Baer, C.A., Ghosh, D. (2007). Module structure of IRBP may provide bases for its complex role in the visual cycle- structure/function study of Xenopus IRBP. BMC. Biochem. 2007, 8:15.
6. Cunningham, L.L., Gonzalez-Fernandez, F. (2003). Internalization of interphotoreceptor retinoid-binding protein (IRBP) by the retinal pigmented epithelium. J. Comp. Neurol. 466:331-342.
7. Loew, A., Gonzalez-Fernandez, F. (2002). Crystal structure of the functional unit of interphotoreceptor retinoid binding protein. Structure 10:43.
8. Cunningham, L.L., Gonzalez-Fernandez, F. (2000) Coordination between production and turnover of interphotoreceptor retinoid-binding protein in zebrafish. Invest. Ophthal Vis Sci. 41:3590.
9. Gonzalez-Fernandez, F., Kaltreider, S.A., Patnaik, B. D., Retief, J.D., Bao,Y. Newman, S., Stoler, M. H., Levine, P.A. (1998). Sebaceous Carcinoma: tumor progression through mutational inactivation of p53. Ophthalmology 105:497.
10. Gonzalez-Fernandez, F., Healy, J.I. (1990). Early expression of the gene for IRBP during photoreceptor differentiation suggests a critical role of the IPM during retinal development. J. Cell Biol. 111:2775.
Teaching
Ocular Pathology Conference – diagnostic surgical pathology, and pathophysiology of ocular disease
Grand Rounds coordinator in Ocular Pathology
Resident Clinical Rotation in Ocular Pathology
Graduate and undergraduate research mentor
Medical Student Clinical Rotation
Medical Student Summer Research Program
Collegiate Science & Technology Entry Program CSTEP/SUNY
Honors/Awards
- The Biology and Diseases of the Posterior Eye Study Section (2006-10).
- Moderator, RPE and Retina Biochemistry; Retinoid-binding proteins (ARVO 2010, 1999, 1997, 1995)
- Chair, Veterans Affairs Institutional Animal Care and Use Committee (2007, 2010-present).
- Invited presentation, Dept of Ophthalmology, College of Medicine, The Catholic University of Korea. Title: “The Visual Cycle: Molecular Pathophysiology” (2009, Seoul, South Korea).
- USA invited reviewer, EVI-GENORET program of the European Union 6th Framework Programme “Functional genomics of the retina in health and disease.” (2009, Paris, France).
- Visiting Professor, Mediterranean Ophthalmology Foundation, and Department of Physiology, Pharmacology & Toxicology, Universidad Cardenal Herrera-CEU, Valencia, Spain (2008-9)
- Keynote presentation, Korean Society of Medical Biochemistry and Molecular Biology. Title: “The vitamin A cycle, a new frontier in vision sciences and medicine” (2009, Yong Pyong, South Korea)
- Visiting Scholar, Institute of Atomic and Molecular Sciences (1998, Sinica, Taipai, Taiwan)
- Editorial Board, Molecular Vision (1995- present)
- NRSA Postdoctoral Fellowship, Neuropathology
- Sigma Xi Research Society Award (doctoral research)
- Mead Johonson Award (doctoral research)
- Pharmacia Travel Fellow (1984, Alicante, Spain)
- Medical Scientist Training Program
- Phi Beta Kappa
- Highest Honors in Biochemistry
- American Institute of Chemists Undergraduate Award.
- Mellon Foundation Undergraduate Research Grant
- Maryland Academy of Science Student Award