Name Steven J. Fliesler
Credentials Ph.D.
Title and Department
Meyer Riwchun Endowed Chair Professor, Vice-Chairman and Director of Research, Department of Ophthalmology
Professor (secondary), Department of Biochemistry
Phone 716-862-6538
Office Address
Buffalo VA Medical Center
Research Service- Bldg 20, Rm 202
3495 Bailey Avenue
Buffalo, NY 14215-1129 U.S.A.
Email Fliesler@buffalo.edu

Education and Training


PhD (biochemistry), Rice University, Houston, TX
Postdoctoral (retinal biochemistry/cell biology),
Cullen Eye Institute, Baylor College of Medicine,
Houston, TX

Research Interest Our lab is focused on studies of retinal degenerations caused by metabolic defects, particularly dyslipidemias involving defective cholesterol metabolism (e.g., Smith-Lemli-Opitz syndrome), using pharmacological and transgenic animal models. Current studies are focused on the role of lipid and protein oxidation in the underlying mechanisms of photoreceptor cell death in such retinal degenerations, using a combination of genomic, proteomic, and lipidomic approaches. We also are studying how allelic apoE isoforms may modulate the severity and duration of retinal degenerations, using transgenic mice that express either human apoE3 or human apoE4 superimposed on an established transgenic mouse model of progressive retinal degeneration. Our lab has established multiple collaborations with vision scientists at other institutions, both domestically and abroad, to pursue these studies, as well as others that involve fundamental aspects of retinal cell biology and biochemistry with relevance to human hereditary retinal degenerations. In recent years, this work has been supported by individual grants from the NEI/NIH (RO1 EY007361; R21 EY017019), the March of Dimes, the Foundation Fighting Blindness (FFB), and both a Senior Scientific Investigator Award as well as an unrestricted departmental grant from Research to Prevent Blindness (RPB).

Selected Publications

1. Fliesler SJ, Peachey NS, Richards MJ, Nagel BA, and Vaughan DK. (2004) Retinal degeneration in a rodent model of Smith-Lemli-Opitz syndrome: Electrophysiological, biochemical, and morphological features. Arch. Ophthalmol. 122, 1190-1200.
2. Vaughan DK, Peachey NS, Richards MJ, Buchan B, and Fliesler SJ (2006) Light-induced exacerbation of retinal degeneration in a rat model of Smith-Lemli-Opitz syndrome. Exp. Eye Res. 82, 496-504.
3. Tserentsoodol N, Sztein J, Campos M, Gordiyenko N, Fariss RN, Lee J-W, Fliesler SJ, and Rodriguez IR. (2006) Uptake of cholesterol by the retina occurs primarily via an LDL-receptor mediated process. Molec. Vis. 12:1306-1318
4. Fliesler SJ, Vaughan DK, Jenewein EC, Richards MJ, Nagel BA, Peachey NS. (2007) Partial rescue of retinal function and sterol steady-state in a rat model of Smith-Lemli-Opitz syndrome. Pediatr. Res. 61(3):273-278.
5. Ford DA, Richards MJ, Brush RS, Anderson RE, and Fliesler SJ (2008) Lipidomic analysis of the retina in a rat model of Smith-Lemli-Opitz syndrome: Alterations in docosahexaenoic acid content of phospholipid molecular species. J. Neurochem, 105(3):1032-47.
6. Boesze-Battaglia K, Damek-Poprawa M, Mitchell DC, Greeley L, Brush RS, Anderson RE, Richards MJ, and Fliesler SJ (2008) Alteration of retinal rod outer segment membrane fluidity in a rat model of Smith-Lemli-Opitz syndrome. J Lipid Res. 49(7):1488-99.
7. Rodriguez IR, Fliesler SJ (2009) Photo-damage generates 7-keto- and 7-hydroxycholesterol in the rat retina via a free radical-mediated mechanism. Photochem. Photobiol. 85(5):1116-1125
8. Cai X, Nash Z, Conley SM, Fliesler SJ, Cooper MJ, Naash MI (2009) A partial structural and functional rescue of a retinitis pigmentosa model with compacted DNA nanoparticles. PLoS One 4(4):e5290.
9. Lintker KB, Kpere-Daibo P, Fliesler SJ, Serfis AB (2009). A comparison of the packing behavior of egg phosphatidylcholine with cholesterol and biogenically related sterols in Langmuir monolayer films. Chem Phys Lipids. 61(1):22-31.
10. Xu L, Liu W, Sheflin LG, Fliesler SJ, Porter NA (2011) Novel oxysterols observed in tissues and fluids of AY9944-treated rats - a model for Smith-Lemli-Opitz Syndrome.  J Lipid Res 51(10): 1810-1820.

Teaching

BMS 501 (Cell Biology); Ophthalmology Resident Lecture Series; Pediatrics Fellowship Lecture Series
Professional Associations ARVO, ISER, ASCB, ASBMB

Awards/Honors

• Research to Prevent Blindness James S. Adams Scholar Award (1992)
• “Scientist of the Year” Award, Saint Louis University Chapter, Sigma Xi (1999)
• Chancellor’s Award in Neuroscience, LSU School of Medicine, New Orleans, LA (2002)
• Research to Prevent Blindness Senior Scientific Investigator Award (2007)
• President, International Society for Eye Research (2012-2014)