Department of Neurology

Faculty Members
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Artur V. Cideciyan, PhD

Research Associate Professor of Ophthalmology
Department: Ophthalmology

Contact information
Scheie Eye Institute
51 N. 39th Street
Philadelphia, PA 19104
Office: 215-662-9986
Fax: 215-662-9388
Education:
B.S. (Mechanical Engineering)
University of Miami, 1986.
M.S. (Biomedical Engineering)
University of Miami, 1988.
Ph.D. (Biomedical Engineering)
University of Miami, 1992.
Post-Graduate Training
Research Associate, Department of Ophthalmology, University of Miami, 1992-1993.
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Description of Research Expertise

Dr. Cideciyan’s primary research interests involve understanding of disease mechanisms in human hereditary retinal degenerations and evaluation of mechanism-specific treatments in these blinding conditions. This is accomplished by the use of non-invasive surrogate measures of biochemical and morphological abnormalities of the retina and the RPE. Knowledge obtained from human retinopathies is also used to glean molecular foundations of normal human vision. Furthermore, studies performed in parallel with non-invasive experimental methods in animal models of these human diseases are used to determine the correspondence between the models and the patients, and pre-clinical therapeutic strategies are tested as appropriate.

Research methods of the laboratory include imaging, electrophysiology and psychophysics as well as mathematical modeling and software development. Optical coherence tomography (OCT) based methods are used to define retinal structure and light scatter characteristics; autofluorescence imaging based methods are used to elucidate RPE abnormalities; electroretinogram (ERG) photoresponses and psychophysical methods are used understand global and local rod and cone photoreceptor dysfunction; and pupillary imaging is used to define post-retinal visual function.

Key Words
Retinal degeneration, photoreceptors, visual cycle, autofluorescence, lipofuscin, melanin, optical coherence tomography, dark adaptation, electroretinography, pupilometry, ABCA4, ABCR, RPE65

Selected Publications

Cideciyan AV, Hauswirth WW, Aleman TS, Kaushal S, Schwartz SB, Boye SL, Windsor EAM, Conlon TJ, Sumaroka A, Roman AJ, Byrne BJ, Jacobson SG. : Vision 1 year after gene therapy for Leber’s congenital amaurosis. New England Journal of Medicine 361: 725-727, 2009.

Cideciyan AV, Hauswirth WW, Aleman TS, Kaushal S, Schwartz SB, Boye SL, Windsor EAM, Conlon TJ, Sumaroka A, Pang J, Roman AJ, Byrne BJ, Jacobson SG.: Human RPE65 gene therapy for Leber congenital amaurosis: persistence of early visual improvements and safety at one year. Human Gene Therapy 20: 999-1004, 2009.

Cideciyan AV, Swider M, Aleman TS, Tsybovsky Y, Schwartz SB, Windsor EA, Roman AJ, Sumaroka A, Steinberg JD, Jacobson SG, Stone EM, Palczewski K. : ABCA4 disease progression and a proposed strategy for gene therapy. Human Molecular Genetics 18: 931-941, 2009.

Cideciyan AV, Aleman TS, Boye SL, Schwartz SB, Kaushal S, Roman AJ, Pang J-j, Sumaroka A, Windsor EAM, Wilson JM, Flotte TR, Fishman GA, Heon E, Stone EM, Byrne BJ, Jacobson SG, Hauswirth WW.: Human gene therapy for RPE65 isomerase deficiency activates the retinoid cycle of vision but with slow rod kenetics. Proceedings of the National Academy of Sciences USA 105: 15112-15117, 2008.

Cideciyan AV, Swider M, Aleman TS, Roman MI, Sumaroka A, Schwartz SB, Stone EM, Jacobson SG.: Reduced-illuminance autofluorescence imaging in ABCA4-associated retinal degenerations. Journal of the Optical Society of America, A 24(5): 1457-67, 2007.

Cideciyan AV, Aleman TS, Jacobson SG, Khanna H, Sumaroka A, Aguirre GK, Schwartz SB, Windsor EAM, He S, Chang B, Stone EM, Swaroop A.: Centrosomal-ciliary gene CEP290/NPHP6 mutations result in blindness with unexpected sparing of photoreceptors and visual brain: implications for therapy of Leber congenital amaurosis. Human Mutation 28: 1074-1083, 2007.

Cideciyan AV, Jacobson SG, Aleman TS, Gu D, Pearce-Kelling SE, Sumaroka A, Acland GM, Aguirre GD.: In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa. Proceedings of the National Academy of Sciences USA 102(14): 5233-8, 2005.

Cideciyan AV, Jacobson SG, Gupta N, Osawa S, Locke KG, Weiss ER, Wright AF, Birch DG, Milam AH. : Cone deactivation kinetics and GRK1/GRK7 expression in enhanced S cone syndrome caused by mutations in NR2E3. Investigative Ophthalmology & Visual Science 44: 1268-1274, 2003.

Cideciyan AV, Haeseleer F, Fariss RN, Aleman TS, Jang G-F, Verlinde CLMJ, Marmor MF, Jacobson SG, Palczewski K.: Rod and cone visual cycle consequences of a null mutation in the 11-cis-retinol dehydrogenase gene in man. Visual Neuroscience 17: 667-678, 2000.

Cideciyan AV and Jacobson SG. : Image analysis of the tapetal-like reflex in heterozygotes of X-linked retinitis pigmentosa. Investigative Ophthalmology & Visual Science. 35: 3812-3824, 1994.

Cideciyan AV, Zhao X, Nielsen L, Khani SC, Jacobson SG, Palczewski K. : Null mutation in the rhodopsin kinase gene slows recovery kinetics of rod and cone phototransduction in man. Proceedings of the National Academy of Sciences USA 95: 328-333, 1998.

Cideciyan AV, Lamb TD, Pugh EN Jr, Huang Y, Jacobson SG. : Rod plateaux during dark adaptation in Sorsby's fundus dystrophy and vitamin A deficiency. Investigative Ophthalmology & Visual Science 38: 1786-1794, 1997.

Cideciyan AV and Mayrovitz HN.: Microcomputer assisted determination of regional myocardial function. Medical & Biological Engineering & Computing 28: 591-594, 1990.

Cideciyan AV, Hood DC, Huang Y, Banin E, Li Z-Y, Stone EM, Milam AH, Jacobson SG.: Disease sequence from mutant rhodopsin allele to rod and cone photoreceptor degeneration in man. Proceedings of the National Academy of Sciences USA 95: 7103-7108, 1998.

Cideciyan AV and Jacobson SG. : An alternative phototransduction model for human rod and cone ERG a-waves: normal parameters and variation with age. Vision Research 36: 2609-2621, 1996.

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Last updated: 10/19/2009
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