Abstract

The health of the retinal pigment epithelium (RPE) can be estimated with autofluorescence (AF) imaging of lipofuscin, which accumulates as a byproduct of retinal exposure to light. Lipofuscin may be toxic to the RPE, and its toxicity may be enhanced by short-wavelength (SW) illumination. The high-intensity and SW excitation light used in conventional AF imaging could, at least in principle, increase the rate of lipofuscin accumulation and/or increase its toxicity. We considered two reduced-illuminance AF imaging (RAFI) methods as alternatives to conventional AF imaging. RAFI methods use either near-infrared (NIR) light or reduced-radiance SW illumination for excitation of fluorophores. We quantified the distribution of RAFI signals in relation to retinal structure and function in patients with the prototypical lipofuscin accumulation disease caused by mutations in ABCA4. There was evidence for two subclinical stages of macular ABCA4 disease involving hyperautofluorescence of both SW- and NIR-RAFI with and without associated loss of visual function. Use of RAFI methods and microperimetry in future clinical trials involving lipofuscinopathies should allow quantification of subclinical disease expression and progression without subjecting the diseased retina/RPE to undue light exposure.

© 2007 Optical Society of America

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2007 (1)

G. H. Travis, M. Golczak, A. R. Moise, and K. Palczewski, "Diseases caused by defects in the visual cycle: retinoids as potential therapeutic agents," Annu. Rev. Pharmacol. Toxicol. 47, 8.1-8.44 (2007).
[CrossRef]

2006 (11)

P. Maiti, J. Kong, S. R. Kim, J. R. Sparrow, R. Allikmets, and R. R. Rando, "Small molecule RPE65 antagonists limit the visual cycle and prevent lipofuscin formation," Biochemistry 45, 852-860 (2006).
[CrossRef] [PubMed]

T. S. Aleman, A. V. Cideciyan, E. A. M. Windsor, S. B. Schwartz, L. M. Gardner, J. M. Emmons, K. G. Duncan, J. D. Steinberg, E. M. Stone, and S. G. Jacobson, "Macular pigment in ABCA4-associated retinal degenerations: response to lutein supplementation," Invest. Ophthalmol. Visual Sci. 47, E-Abstract 5810 (2006).

J. Hopkins, A. Walsh, and U. Chakravarthy, "Fundus autofluorescence in age-related macular degeneration: an epiphenomenon?" Invest. Ophthalmol. Visual Sci. 47, 2269-2271 (2006).
[CrossRef]

D. M. Paskowitz, M. M. Lavail, and J. L. Duncan, "Light and inherited retinal degeneration," Br. J. Ophthamol. 90, 1060-1066 (2006).
[CrossRef]

F. C. Delori, D. G. Goger, C. Keilhauer, P. Salvetti, and G. Staurenghi, "Bimodal spatial distribution of macular pigment: evidence of a gender relationship," J. Opt. Soc. Am. A 23, 521-538 (2006).
[CrossRef]

S. Schmitz-Valckenberg, A. Bindewald-Wittich, J. Dolar-Szczasny, J. Dreyhaupt, S. Wolf, H. P. Scholl, F. G. Holz, and Fundus Autofluorescence in Age-Related Macular Degeneration Study Group, "Correlation between the area of increased autofluorescence surrounding geographic atrophy and disease progression in patients with AMD," Invest. Ophthalmol. Visual Sci. 47, 2648-2654 (2006).
[CrossRef]

J. C. Hwang, J. W. Chan, S. Chang, and R. T. Smith, "Predictive value of fundus autofluorescence for development of geographic atrophy in age-related macular degeneration," Invest. Ophthalmol. Visual Sci. 47, 2655-2661 (2006).
[CrossRef]

J. S. Sunness, M. D. Ziegler, and C. A. Applegate, "Issues in quantifying atrophic macular disease using retinal autofluorescence," Retina 26, 666-672 (2006).
[CrossRef] [PubMed]

A. W. Weinberger, A. Lappas, T. Kirschkamp, B. A. Mazinani, J. K. Huth, B. Mohammadi, and P. Walter, "Fundus near infrared fluorescence correlates with fundus near infrared reflectance," Invest. Ophthalmol. Visual Sci. 47, 3098-3108 (2006).
[CrossRef]

C. N. Keilhauer and F. C. Delori, "Near-infrared autofluorescence imaging of the fundus: visualization of ocular melanin," Invest. Ophthalmol. Visual Sci. 47, 3556-3564 (2006).
[CrossRef]

S. G. Jacobson, A. V. Cideciyan, A. Sumaroka, T. S. Aleman, S. B. Schwartz, E. A. M. Windsor, A. J. Roman, E. M. Stone, and I. M. MacDonald, "Remodeling of the human retina in choroideremia--Rab Escort Protein 1 (REP-1) mutations," Invest. Ophthalmol. Visual Sci. 47, 4113-4120 (2006).
[CrossRef]

2005 (8)

A. V. Cideciyan, M. Swider, T. S. Aleman, A. Sumaroka, S. B. Schwartz, M. I. Roman, A. H. Milam, J. Bennett, E. M. Stone, and S. G. Jacobson, "ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina," Invest. Ophthalmol. Visual Sci. 46, 4739-4746 (2005).
[CrossRef]

A. V. Cideciyan, S. G. Jacobson, T. S. Aleman, D. Gu, S. E. Pearce-Kelling, A. Sumaroka, G. M. Acland, and G. D. Aguirre, "In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa," Proc. Natl. Acad. Sci. U.S.A. 102, 5233-5238 (2005).
[CrossRef] [PubMed]

S. G. Jacobson, T. S. Aleman, A. V. Cideciyan, A. Sumaroka, S. B. Schwartz, E. A. M. Windsor, E. I. Traboulsi, E. Heon, S. J. Pittler, A. H. Milam, A. M. Maguire, K. Palczewski, E. M. Stone, and J. Bennett, "Identifying photoreceptors in blind eyes due to RPE65 mutations: prerequisite for human gene therapy success," Proc. Natl. Acad. Sci. U.S.A. 102, 6177-6182 (2005).
[CrossRef] [PubMed]

J. R. Sparrow and M. Boulton, "RPE lipofuscin and its role in retinal pathobiology," Exp. Eye Res. 80, 595-606 (2005).
[CrossRef] [PubMed]

J. J. Jorzik, A. Bindewald, S. Dithmar, and F. G. Holz, "Digital simultaneous fluorescein and indocyanine green angiography, autofluorescence, and red-free imaging with a solid-state laser-based confocal scanning laser ophthalmoscope," Retina 25, 405-416 (2005).
[CrossRef] [PubMed]

G. A. Fishman, S. G. Jacobson, K. R. Alexander, A. V. Cideciyan, D. G. Birch, R. G. Weleber, and D. C. Hood, "Outcome measures and their application in clinical trials for retinal degenerative disease: outline, review, and perspective," Retina 25, 772-777 (2005).
[CrossRef] [PubMed]

D. Sliney, D. Aron-Rosa, F. Delori, F. Fankhauser, R. Landry, M. Mainster, J. Marshall, B. Rassow, B. Stuck, S. Trokel, T. M. West, M. Wolffe, and International Commission on Non-Ionizing Radiation Protection, "Adjustment of guidelines for exposure of the eye to optical radiation from ocular instruments: statement from a task group of the International Commission on Non-Ionizing Radiation Protection (ICNIRP)," Appl. Opt. 44, 2162-2176 (2005).
[CrossRef]

M. Golczak, V. Kuksa, T. Maeda, A. R. Moise, and K. Palczewski, "Positively charged retinoids are potent and selective inhibitors of the trans-cis isomerization in the retinoid (visual) cycle," Proc. Natl. Acad. Sci. U.S.A. 102, 8162-8167 (2005).
[CrossRef] [PubMed]

2004 (5)

D. Gibbs, S. M. Azarian, C. Lillo, J. Kitamoto, A. E. Klomp, K. P. Steel, R. T. Libby, and D. S. Williams, "Role of myosin VIIa and Rab27a in the motility and localization of RPE melanosomes," J. Cell. Sci. 117, 6473-6483 (2004).
[CrossRef]

N. Lois, A. S. Halfyard, A. C. Bird, G. E. Holder, and F. W. Fitzke, "Fundus autofluorescence in Stargardt macular dystrophy--fundus flavimaculatus," Am. J. Ophthalmol. 138, 55-63 (2004).
[CrossRef] [PubMed]

A. V. Cideciyan, T. S. Aleman, M. Swider, S. B. Schwartz, J. D. Steinberg, A. J. Brucker, A. M. Maguire, J. Bennett, E. M. Stone, and S. G. Jacobson, "Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence," Hum. Mol. Genet. 13, 525-534 (2004).
[CrossRef] [PubMed]

M. Boulton, M. Rozanowska, B. Rozanowski, and T. Wess, "The photoreactivity of ocular lipofuscin," Photochem. Photobiol. Sci. 3, 759-764 (2004).
[CrossRef] [PubMed]

S. Herlidou, R. Grebe, F. Grados, N. Leuyer, P. Fardellone, and M. E. Meyer, "Influence of age and osteoporosis on calcaneus trabecular bone structure: a preliminary in vivo MRI study by quantitative texture analysis," Magn. Reson. Imaging 22, 237-243 (2004).
[CrossRef] [PubMed]

2003 (4)

R. A. Radu, N. L. Mata, S. Nusinowitz, X. Liu, P. A. Sieving, and G. H. Travis, "Treatment with isotretinoin inhibits lipofuscin accumulation in a mouse model of recessive Stargardt's macular degeneration," Proc. Natl. Acad. Sci. U.S.A. 100, 4742-4747 (2003).
[CrossRef] [PubMed]

R. F. Spaide, "Fundus autofluorescence and age-related macular degeneration," Ophthalmology 110, 392-329 (2003).
[CrossRef] [PubMed]

G. A. Fishman, E. M. Stone, D. A. Eliason, C. M. Taylor, M. Lindeman, and D. J. Derlacki, "ABCA4 gene sequence variations in patients with autosomal recessive cone-rod dystrophy," Arch. Ophthalmol. (Chicago) 121, 851-855 (2003).

N. Fishkin, Y. P. Jang, Y. Itagaki, J. R. Sparrow, and K. Nakanishi, "A2-rhodopsin: a new fluorophore isolated from photoreceptor outer segments," Org. Biomol. Chem. 1, 1101-1105 (2003).
[CrossRef] [PubMed]

2002 (1)

C. Gerth, M. Andrassi-Darida, M. Bock, M. N. Preising, B. H. Weber, and B. Lorenz, "Phenotypes of 16 Stargardt macular dystrophy/fundus flavimaculatus patients with known ABCA4 mutations and evaluation of genotype-phenotype correlation," Graefes Arch. Clin. Exp. Ophthalmol. 240, 628-638 (2002).
[CrossRef] [PubMed]

2001 (6)

F. C. Delori, D. G. Goger, and C. K. Dorey, "Age-related accumulation and spatial distribution of lipofuscin in RPE of normal subjects," Invest. Ophthalmol. Visual Sci. 42, 1855-1866 (2001).

F. G. Holz, C. Bellman, S. Staudt, F. Schutt, and H. E. Volcker, "Fundus autofluorescence and development of geographic atrophy in age-related macular degeneration," Invest. Ophthalmol. Visual Sci. 42, 1051-1056 (2001).

N. Lois, G. E. Holder, C. Bunce, F. W. Fitzke, and A. C. Bird, "Phenotypic subtypes of Stargardt macular dystrophy--fundus flavimaculatus," Arch. Ophthalmol. (Chicago) 119, 359-369 (2001).

P. A. Sieving, P. Chaudhry, M. Kondo, M. Provenzano, D. Wu, T. J. Carlson, R. A. Bush, and D. A. Thompson, "Inhibition of the visual cycle in vivo by 13-cis retinoic acid protects from light damage and provides a mechanism for night blindness in isotretinoin therapy," Proc. Natl. Acad. Sci. U.S.A. 98, 1835-1840 (2001).
[CrossRef] [PubMed]

A. R. Webster, E. Heon, A. J. Lotery, K. Vandenburgh, T. L. Casavant, K. T. Oh, G. Beck, G. A. Fishman, B. L. Lam, A. Levin, J. R. Heckenlively, S. G. Jacobson, R. G. Weleber, V. C. Sheffield, and E. M. Stone, "An analysis of allelic variation in the ABCA4 gene," Invest. Ophthalmol. Visual Sci. 42, 1179-1189 (2001).

P. Kayatz, G. Thumann, T. T. Luther, J. F. Jordan, K. U. Bartz-Schmidt, P. J. Esser, and U. Schraermeyer, "Oxidation causes melanin fluorescence," Invest. Ophthalmol. Visual Sci. 42, 241-246 (2001).

1999 (3)

G. A. Fishman, E. M. Stone, S. Grover, D. J. Derlacki, H. L. Haines, and R. R. Hockey, "Variation of clinical expression in patients with Stargardt dystrophy and sequence variations in the ABCR gene," Arch. Ophthalmol. (Chicago) 117, 504-510 (1999).

J. Weng, N. L. Mata, S. M. Azarian, R. T. Tzekov, D. G. Birch, and G. H. Travis, "Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice," Cell 98, 13-23 (1999).
[CrossRef] [PubMed]

F. G. Holz, C. Bellmann, M. Margaritidis, F. Schutt, T. P. Otto, and H. E. Volcker, "Patterns of increased in vivo fundus autofluorescence in the junctional zone of geographic atrophy of the retinal pigment epithelium associated with age-related macular degeneration," Graefe's Arch. Clin. Exp. Ophthalmol. 237, 145-152 (1999).
[CrossRef]

1998 (3)

Y. Huang, A. V. Cideciyan, G. I. Papastergiou, E. Banin, S. L. Semple-Rowland, A. H. Milam, and S. G. Jacobson, "Relation of optical coherence tomography to microanatomy in normal and rd chickens," Invest. Ophthalmol. Visual Sci. 39, 2405-2416 (1998).

A. Martinez-Mir, E. Paloma, R. Allikmets, C. Ayuso, T. del Rio, M. Dean, L. Vilageliu, R. Gonzalez-Duarte, and S. Balcells, "Retinitis pigmentosa caused by a homozygous mutation in the Stargardt disease gene ABCR," Nat. Genet. 18, 11-12 (1998).
[CrossRef] [PubMed]

F. P. Cremers, D. J. van de Pol, M. van Driel, A. I. den Hollander, F. J. van Haren, N. V. Knoers, N. Tijmes, A. A. Bergen, K. Rohrschneider, A. Blankenagel, A. J. Pinckers, A. F. Deutman, and C. B. Hoyng, "Autosomal recessive retinitis pigmentosa and cone-rod dystrophy caused by splice site mutations in the Stargardt's disease gene ABCR," Hum. Mol. Genet. 7, 355-362 (1998).
[CrossRef] [PubMed]

1997 (2)

R. Allikmets, N. Singh, H. Sun, N. F. Shroyer, A. Hutchinson, A. Chidambaram, B. Gerrard, L. Baird, D. Stauffer, A. Peiffer, A. Rattner, P. Smallwood, Y. Li, K. L. Anderson, R. A. Lewis, J. Nathans, M. Leppert, M. Dean, and J. R. Lupski, "A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy," Nat. Genet. 15, 236-246 (1997).
[CrossRef] [PubMed]

C. Bellmann, F. G. Holz, O. Schapp, H. E. Volcker, and T. P. Otto, "[Topography of fundus autofluorescence with a new confocal scanning laser ophthalmoscope]," (in German) Ophthalmologe 94, 385-391 (1997).
[CrossRef] [PubMed]

1996 (2)

A. E. Elsner, S. A. Burns, J. J. Weiter, and F. C. Delori, "Infrared imaging of sub-retinal structures in the human ocular fundus," Vision Res. 36, 191-205 (1996).
[CrossRef] [PubMed]

F. C. Piccolino, L. Borgia, E. Zinicola, M. Iester, and S. Torrielli, "Pre-injection fluorescence in indocyanine green angiography," Ophthalmology 103, 1837-1845 (1996).
[PubMed]

1995 (3)

A. von Ruckmann, F. W. Fitzke, and A. C. Bird, "Distribution of fundus autofluorescence with a scanning laser ophthalmoscope," Br. J. Ophthamol. 79, 407-412 (1995).
[CrossRef]

F. C. Delori, C. K. Dorey, G. Staurenghi, O. Arend, D. G. Goger, and J. J. Weiter, "In vivo fluorescence of the ocular fundus exhibits retinal pigment epithelium lipofuscin characteristics," Invest. Ophthalmol. Visual Sci. 36, 718-729 (1995).

F. C. Delori, G. Staurenghi, O. Arend, C. K. Dorey, D. G. Goger, and J. J. Weiter, "In vivo measurement of lipofuscin in Stargardt's disease--fundus flavimaculatus," Invest. Ophthalmol. Visual Sci. 36, 2327-2331 (1995).

1994 (2)

F. C. Delori, "Spectrophotometer for noninvasive measurement of intrinsic fluorescence and reflectance of ocular fundus," Appl. Opt. 33, 7439-7452 (1994).
[CrossRef] [PubMed]

C. D. Birnbach, M. Jarvelainen, D. E. Possin, and A. H. Milam, "Histopathology and immunocytochemistry of the neurosensory retina in fundus flavimaculatus," Ophthalmology 101, 1211-1219 (1994).
[PubMed]

1993 (2)

U. Schraermeyer, "Does melanin turnover occur in the eyes of adult vertebrates?" Pigment Cell Res. 6, 193-204 (1993).
[CrossRef]

G. E. Eldred and M. R. Lasky, "Retinal age pigments generated by self-assembling lysosomotropic detergents," Nature 361, 724-726 (1993).
[CrossRef] [PubMed]

1989 (2)

K. Kitagawa, S. Nishida, and Y. Ogura, "In vivo quantitation of autofluorescence in human retinal pigment epithelium," Ophthalmologica 199, 116-121 (1989).
[CrossRef] [PubMed]

R. W. Knighton, S. G. Jacobson, and C. M. Kemp, "The spectral reflectance of the nerve fiber layer of the macaque retina," Invest. Ophthalmol. Visual Sci. 30, 2392-2402 (1989).

1988 (2)

M. Iwasaki and H. Inomata, "Lipofuscin granules in human photoreceptor cells," Invest. Ophthalmol. Visual Sci. 29, 671-679 (1988).

G. E. Eldred and M. L. Katz, "Fluorophores of the human retinal pigment epithelium: separation and spectral characterization," Exp. Eye Res. 47, 71-86 (1988).
[CrossRef]

1986 (2)

J. J. Weiter, F. C. Delori, G. L. Wing, and K. A. Fitch, "Retinal pigment epithelial lipofuscin and melanin and choroidal melanin in human eyes," Invest. Ophthalmol. Visual Sci. 27, 145-152 (1986).

G. S. Tucker, "Refractile bodies in the inner segments of cones in the aging human retina," Invest. Ophthalmol. Visual Sci. 27, 708-715 (1986).

1980 (1)

F. C. Delori, J. S. Parker, and M. A. Mainster, "Light levels in fundus photography and fluorescein angiography," Vision Res. 20, 1099-1104 (1980).
[CrossRef] [PubMed]

1978 (1)

L. Feeney, "Lipofuscin and melanin of human retinal pigment epithelium. Fluorescence, enzyme cytochemical, and ultrastructural studies," Invest. Ophthalmol. Visual Sci. 17, 583-600 (1978).

1975 (1)

M. M. Galloway, "Texture analysis using gray level run-lengths," Comput. Graph. Image Process. 4, 172-179 (1975).
[CrossRef]

Acland, G. M.

A. V. Cideciyan, S. G. Jacobson, T. S. Aleman, D. Gu, S. E. Pearce-Kelling, A. Sumaroka, G. M. Acland, and G. D. Aguirre, "In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa," Proc. Natl. Acad. Sci. U.S.A. 102, 5233-5238 (2005).
[CrossRef] [PubMed]

Aguirre, G. D.

A. V. Cideciyan, S. G. Jacobson, T. S. Aleman, D. Gu, S. E. Pearce-Kelling, A. Sumaroka, G. M. Acland, and G. D. Aguirre, "In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa," Proc. Natl. Acad. Sci. U.S.A. 102, 5233-5238 (2005).
[CrossRef] [PubMed]

Aleman, T. S.

S. G. Jacobson, A. V. Cideciyan, A. Sumaroka, T. S. Aleman, S. B. Schwartz, E. A. M. Windsor, A. J. Roman, E. M. Stone, and I. M. MacDonald, "Remodeling of the human retina in choroideremia--Rab Escort Protein 1 (REP-1) mutations," Invest. Ophthalmol. Visual Sci. 47, 4113-4120 (2006).
[CrossRef]

T. S. Aleman, A. V. Cideciyan, E. A. M. Windsor, S. B. Schwartz, L. M. Gardner, J. M. Emmons, K. G. Duncan, J. D. Steinberg, E. M. Stone, and S. G. Jacobson, "Macular pigment in ABCA4-associated retinal degenerations: response to lutein supplementation," Invest. Ophthalmol. Visual Sci. 47, E-Abstract 5810 (2006).

S. G. Jacobson, T. S. Aleman, A. V. Cideciyan, A. Sumaroka, S. B. Schwartz, E. A. M. Windsor, E. I. Traboulsi, E. Heon, S. J. Pittler, A. H. Milam, A. M. Maguire, K. Palczewski, E. M. Stone, and J. Bennett, "Identifying photoreceptors in blind eyes due to RPE65 mutations: prerequisite for human gene therapy success," Proc. Natl. Acad. Sci. U.S.A. 102, 6177-6182 (2005).
[CrossRef] [PubMed]

A. V. Cideciyan, S. G. Jacobson, T. S. Aleman, D. Gu, S. E. Pearce-Kelling, A. Sumaroka, G. M. Acland, and G. D. Aguirre, "In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa," Proc. Natl. Acad. Sci. U.S.A. 102, 5233-5238 (2005).
[CrossRef] [PubMed]

A. V. Cideciyan, M. Swider, T. S. Aleman, A. Sumaroka, S. B. Schwartz, M. I. Roman, A. H. Milam, J. Bennett, E. M. Stone, and S. G. Jacobson, "ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina," Invest. Ophthalmol. Visual Sci. 46, 4739-4746 (2005).
[CrossRef]

A. V. Cideciyan, T. S. Aleman, M. Swider, S. B. Schwartz, J. D. Steinberg, A. J. Brucker, A. M. Maguire, J. Bennett, E. M. Stone, and S. G. Jacobson, "Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence," Hum. Mol. Genet. 13, 525-534 (2004).
[CrossRef] [PubMed]

Alexander, K. R.

G. A. Fishman, S. G. Jacobson, K. R. Alexander, A. V. Cideciyan, D. G. Birch, R. G. Weleber, and D. C. Hood, "Outcome measures and their application in clinical trials for retinal degenerative disease: outline, review, and perspective," Retina 25, 772-777 (2005).
[CrossRef] [PubMed]

Allikmets, R.

P. Maiti, J. Kong, S. R. Kim, J. R. Sparrow, R. Allikmets, and R. R. Rando, "Small molecule RPE65 antagonists limit the visual cycle and prevent lipofuscin formation," Biochemistry 45, 852-860 (2006).
[CrossRef] [PubMed]

A. Martinez-Mir, E. Paloma, R. Allikmets, C. Ayuso, T. del Rio, M. Dean, L. Vilageliu, R. Gonzalez-Duarte, and S. Balcells, "Retinitis pigmentosa caused by a homozygous mutation in the Stargardt disease gene ABCR," Nat. Genet. 18, 11-12 (1998).
[CrossRef] [PubMed]

R. Allikmets, N. Singh, H. Sun, N. F. Shroyer, A. Hutchinson, A. Chidambaram, B. Gerrard, L. Baird, D. Stauffer, A. Peiffer, A. Rattner, P. Smallwood, Y. Li, K. L. Anderson, R. A. Lewis, J. Nathans, M. Leppert, M. Dean, and J. R. Lupski, "A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy," Nat. Genet. 15, 236-246 (1997).
[CrossRef] [PubMed]

Anderson, K. L.

R. Allikmets, N. Singh, H. Sun, N. F. Shroyer, A. Hutchinson, A. Chidambaram, B. Gerrard, L. Baird, D. Stauffer, A. Peiffer, A. Rattner, P. Smallwood, Y. Li, K. L. Anderson, R. A. Lewis, J. Nathans, M. Leppert, M. Dean, and J. R. Lupski, "A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy," Nat. Genet. 15, 236-246 (1997).
[CrossRef] [PubMed]

Andrassi-Darida, M.

C. Gerth, M. Andrassi-Darida, M. Bock, M. N. Preising, B. H. Weber, and B. Lorenz, "Phenotypes of 16 Stargardt macular dystrophy/fundus flavimaculatus patients with known ABCA4 mutations and evaluation of genotype-phenotype correlation," Graefes Arch. Clin. Exp. Ophthalmol. 240, 628-638 (2002).
[CrossRef] [PubMed]

Applegate, C. A.

J. S. Sunness, M. D. Ziegler, and C. A. Applegate, "Issues in quantifying atrophic macular disease using retinal autofluorescence," Retina 26, 666-672 (2006).
[CrossRef] [PubMed]

Arend, O.

F. C. Delori, C. K. Dorey, G. Staurenghi, O. Arend, D. G. Goger, and J. J. Weiter, "In vivo fluorescence of the ocular fundus exhibits retinal pigment epithelium lipofuscin characteristics," Invest. Ophthalmol. Visual Sci. 36, 718-729 (1995).

F. C. Delori, G. Staurenghi, O. Arend, C. K. Dorey, D. G. Goger, and J. J. Weiter, "In vivo measurement of lipofuscin in Stargardt's disease--fundus flavimaculatus," Invest. Ophthalmol. Visual Sci. 36, 2327-2331 (1995).

Aron-Rosa, D.

Ayuso, C.

A. Martinez-Mir, E. Paloma, R. Allikmets, C. Ayuso, T. del Rio, M. Dean, L. Vilageliu, R. Gonzalez-Duarte, and S. Balcells, "Retinitis pigmentosa caused by a homozygous mutation in the Stargardt disease gene ABCR," Nat. Genet. 18, 11-12 (1998).
[CrossRef] [PubMed]

Azarian, S. M.

D. Gibbs, S. M. Azarian, C. Lillo, J. Kitamoto, A. E. Klomp, K. P. Steel, R. T. Libby, and D. S. Williams, "Role of myosin VIIa and Rab27a in the motility and localization of RPE melanosomes," J. Cell. Sci. 117, 6473-6483 (2004).
[CrossRef]

J. Weng, N. L. Mata, S. M. Azarian, R. T. Tzekov, D. G. Birch, and G. H. Travis, "Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice," Cell 98, 13-23 (1999).
[CrossRef] [PubMed]

Baird, L.

R. Allikmets, N. Singh, H. Sun, N. F. Shroyer, A. Hutchinson, A. Chidambaram, B. Gerrard, L. Baird, D. Stauffer, A. Peiffer, A. Rattner, P. Smallwood, Y. Li, K. L. Anderson, R. A. Lewis, J. Nathans, M. Leppert, M. Dean, and J. R. Lupski, "A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy," Nat. Genet. 15, 236-246 (1997).
[CrossRef] [PubMed]

Balcells, S.

A. Martinez-Mir, E. Paloma, R. Allikmets, C. Ayuso, T. del Rio, M. Dean, L. Vilageliu, R. Gonzalez-Duarte, and S. Balcells, "Retinitis pigmentosa caused by a homozygous mutation in the Stargardt disease gene ABCR," Nat. Genet. 18, 11-12 (1998).
[CrossRef] [PubMed]

Banin, E.

Y. Huang, A. V. Cideciyan, G. I. Papastergiou, E. Banin, S. L. Semple-Rowland, A. H. Milam, and S. G. Jacobson, "Relation of optical coherence tomography to microanatomy in normal and rd chickens," Invest. Ophthalmol. Visual Sci. 39, 2405-2416 (1998).

Bartz-Schmidt, K. U.

P. Kayatz, G. Thumann, T. T. Luther, J. F. Jordan, K. U. Bartz-Schmidt, P. J. Esser, and U. Schraermeyer, "Oxidation causes melanin fluorescence," Invest. Ophthalmol. Visual Sci. 42, 241-246 (2001).

Beck, G.

A. R. Webster, E. Heon, A. J. Lotery, K. Vandenburgh, T. L. Casavant, K. T. Oh, G. Beck, G. A. Fishman, B. L. Lam, A. Levin, J. R. Heckenlively, S. G. Jacobson, R. G. Weleber, V. C. Sheffield, and E. M. Stone, "An analysis of allelic variation in the ABCA4 gene," Invest. Ophthalmol. Visual Sci. 42, 1179-1189 (2001).

Bellman, C.

F. G. Holz, C. Bellman, S. Staudt, F. Schutt, and H. E. Volcker, "Fundus autofluorescence and development of geographic atrophy in age-related macular degeneration," Invest. Ophthalmol. Visual Sci. 42, 1051-1056 (2001).

Bellmann, C.

F. G. Holz, C. Bellmann, M. Margaritidis, F. Schutt, T. P. Otto, and H. E. Volcker, "Patterns of increased in vivo fundus autofluorescence in the junctional zone of geographic atrophy of the retinal pigment epithelium associated with age-related macular degeneration," Graefe's Arch. Clin. Exp. Ophthalmol. 237, 145-152 (1999).
[CrossRef]

C. Bellmann, F. G. Holz, O. Schapp, H. E. Volcker, and T. P. Otto, "[Topography of fundus autofluorescence with a new confocal scanning laser ophthalmoscope]," (in German) Ophthalmologe 94, 385-391 (1997).
[CrossRef] [PubMed]

Bennett, J.

S. G. Jacobson, T. S. Aleman, A. V. Cideciyan, A. Sumaroka, S. B. Schwartz, E. A. M. Windsor, E. I. Traboulsi, E. Heon, S. J. Pittler, A. H. Milam, A. M. Maguire, K. Palczewski, E. M. Stone, and J. Bennett, "Identifying photoreceptors in blind eyes due to RPE65 mutations: prerequisite for human gene therapy success," Proc. Natl. Acad. Sci. U.S.A. 102, 6177-6182 (2005).
[CrossRef] [PubMed]

A. V. Cideciyan, M. Swider, T. S. Aleman, A. Sumaroka, S. B. Schwartz, M. I. Roman, A. H. Milam, J. Bennett, E. M. Stone, and S. G. Jacobson, "ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina," Invest. Ophthalmol. Visual Sci. 46, 4739-4746 (2005).
[CrossRef]

A. V. Cideciyan, T. S. Aleman, M. Swider, S. B. Schwartz, J. D. Steinberg, A. J. Brucker, A. M. Maguire, J. Bennett, E. M. Stone, and S. G. Jacobson, "Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence," Hum. Mol. Genet. 13, 525-534 (2004).
[CrossRef] [PubMed]

Bergen, A. A.

F. P. Cremers, D. J. van de Pol, M. van Driel, A. I. den Hollander, F. J. van Haren, N. V. Knoers, N. Tijmes, A. A. Bergen, K. Rohrschneider, A. Blankenagel, A. J. Pinckers, A. F. Deutman, and C. B. Hoyng, "Autosomal recessive retinitis pigmentosa and cone-rod dystrophy caused by splice site mutations in the Stargardt's disease gene ABCR," Hum. Mol. Genet. 7, 355-362 (1998).
[CrossRef] [PubMed]

Bindewald, A.

J. J. Jorzik, A. Bindewald, S. Dithmar, and F. G. Holz, "Digital simultaneous fluorescein and indocyanine green angiography, autofluorescence, and red-free imaging with a solid-state laser-based confocal scanning laser ophthalmoscope," Retina 25, 405-416 (2005).
[CrossRef] [PubMed]

Bindewald-Wittich, A.

S. Schmitz-Valckenberg, A. Bindewald-Wittich, J. Dolar-Szczasny, J. Dreyhaupt, S. Wolf, H. P. Scholl, F. G. Holz, and Fundus Autofluorescence in Age-Related Macular Degeneration Study Group, "Correlation between the area of increased autofluorescence surrounding geographic atrophy and disease progression in patients with AMD," Invest. Ophthalmol. Visual Sci. 47, 2648-2654 (2006).
[CrossRef]

Birch, D. G.

G. A. Fishman, S. G. Jacobson, K. R. Alexander, A. V. Cideciyan, D. G. Birch, R. G. Weleber, and D. C. Hood, "Outcome measures and their application in clinical trials for retinal degenerative disease: outline, review, and perspective," Retina 25, 772-777 (2005).
[CrossRef] [PubMed]

J. Weng, N. L. Mata, S. M. Azarian, R. T. Tzekov, D. G. Birch, and G. H. Travis, "Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice," Cell 98, 13-23 (1999).
[CrossRef] [PubMed]

Bird, A. C.

N. Lois, A. S. Halfyard, A. C. Bird, G. E. Holder, and F. W. Fitzke, "Fundus autofluorescence in Stargardt macular dystrophy--fundus flavimaculatus," Am. J. Ophthalmol. 138, 55-63 (2004).
[CrossRef] [PubMed]

N. Lois, G. E. Holder, C. Bunce, F. W. Fitzke, and A. C. Bird, "Phenotypic subtypes of Stargardt macular dystrophy--fundus flavimaculatus," Arch. Ophthalmol. (Chicago) 119, 359-369 (2001).

A. von Ruckmann, F. W. Fitzke, and A. C. Bird, "Distribution of fundus autofluorescence with a scanning laser ophthalmoscope," Br. J. Ophthamol. 79, 407-412 (1995).
[CrossRef]

Birnbach, C. D.

C. D. Birnbach, M. Jarvelainen, D. E. Possin, and A. H. Milam, "Histopathology and immunocytochemistry of the neurosensory retina in fundus flavimaculatus," Ophthalmology 101, 1211-1219 (1994).
[PubMed]

Blankenagel, A.

F. P. Cremers, D. J. van de Pol, M. van Driel, A. I. den Hollander, F. J. van Haren, N. V. Knoers, N. Tijmes, A. A. Bergen, K. Rohrschneider, A. Blankenagel, A. J. Pinckers, A. F. Deutman, and C. B. Hoyng, "Autosomal recessive retinitis pigmentosa and cone-rod dystrophy caused by splice site mutations in the Stargardt's disease gene ABCR," Hum. Mol. Genet. 7, 355-362 (1998).
[CrossRef] [PubMed]

Bock, M.

C. Gerth, M. Andrassi-Darida, M. Bock, M. N. Preising, B. H. Weber, and B. Lorenz, "Phenotypes of 16 Stargardt macular dystrophy/fundus flavimaculatus patients with known ABCA4 mutations and evaluation of genotype-phenotype correlation," Graefes Arch. Clin. Exp. Ophthalmol. 240, 628-638 (2002).
[CrossRef] [PubMed]

Borgia, L.

F. C. Piccolino, L. Borgia, E. Zinicola, M. Iester, and S. Torrielli, "Pre-injection fluorescence in indocyanine green angiography," Ophthalmology 103, 1837-1845 (1996).
[PubMed]

Boulton, M.

J. R. Sparrow and M. Boulton, "RPE lipofuscin and its role in retinal pathobiology," Exp. Eye Res. 80, 595-606 (2005).
[CrossRef] [PubMed]

M. Boulton, M. Rozanowska, B. Rozanowski, and T. Wess, "The photoreactivity of ocular lipofuscin," Photochem. Photobiol. Sci. 3, 759-764 (2004).
[CrossRef] [PubMed]

Brucker, A. J.

A. V. Cideciyan, T. S. Aleman, M. Swider, S. B. Schwartz, J. D. Steinberg, A. J. Brucker, A. M. Maguire, J. Bennett, E. M. Stone, and S. G. Jacobson, "Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence," Hum. Mol. Genet. 13, 525-534 (2004).
[CrossRef] [PubMed]

Bunce, C.

N. Lois, G. E. Holder, C. Bunce, F. W. Fitzke, and A. C. Bird, "Phenotypic subtypes of Stargardt macular dystrophy--fundus flavimaculatus," Arch. Ophthalmol. (Chicago) 119, 359-369 (2001).

Burns, S. A.

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A. V. Cideciyan, S. G. Jacobson, T. S. Aleman, D. Gu, S. E. Pearce-Kelling, A. Sumaroka, G. M. Acland, and G. D. Aguirre, "In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa," Proc. Natl. Acad. Sci. U.S.A. 102, 5233-5238 (2005).
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G. A. Fishman, E. M. Stone, S. Grover, D. J. Derlacki, H. L. Haines, and R. R. Hockey, "Variation of clinical expression in patients with Stargardt dystrophy and sequence variations in the ABCR gene," Arch. Ophthalmol. (Chicago) 117, 504-510 (1999).

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N. Lois, A. S. Halfyard, A. C. Bird, G. E. Holder, and F. W. Fitzke, "Fundus autofluorescence in Stargardt macular dystrophy--fundus flavimaculatus," Am. J. Ophthalmol. 138, 55-63 (2004).
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S. G. Jacobson, T. S. Aleman, A. V. Cideciyan, A. Sumaroka, S. B. Schwartz, E. A. M. Windsor, E. I. Traboulsi, E. Heon, S. J. Pittler, A. H. Milam, A. M. Maguire, K. Palczewski, E. M. Stone, and J. Bennett, "Identifying photoreceptors in blind eyes due to RPE65 mutations: prerequisite for human gene therapy success," Proc. Natl. Acad. Sci. U.S.A. 102, 6177-6182 (2005).
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A. V. Cideciyan, M. Swider, T. S. Aleman, A. Sumaroka, S. B. Schwartz, M. I. Roman, A. H. Milam, J. Bennett, E. M. Stone, and S. G. Jacobson, "ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina," Invest. Ophthalmol. Visual Sci. 46, 4739-4746 (2005).
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F. G. Holz, C. Bellmann, M. Margaritidis, F. Schutt, T. P. Otto, and H. E. Volcker, "Patterns of increased in vivo fundus autofluorescence in the junctional zone of geographic atrophy of the retinal pigment epithelium associated with age-related macular degeneration," Graefe's Arch. Clin. Exp. Ophthalmol. 237, 145-152 (1999).
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S. G. Jacobson, A. V. Cideciyan, A. Sumaroka, T. S. Aleman, S. B. Schwartz, E. A. M. Windsor, A. J. Roman, E. M. Stone, and I. M. MacDonald, "Remodeling of the human retina in choroideremia--Rab Escort Protein 1 (REP-1) mutations," Invest. Ophthalmol. Visual Sci. 47, 4113-4120 (2006).
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A. R. Webster, E. Heon, A. J. Lotery, K. Vandenburgh, T. L. Casavant, K. T. Oh, G. Beck, G. A. Fishman, B. L. Lam, A. Levin, J. R. Heckenlively, S. G. Jacobson, R. G. Weleber, V. C. Sheffield, and E. M. Stone, "An analysis of allelic variation in the ABCA4 gene," Invest. Ophthalmol. Visual Sci. 42, 1179-1189 (2001).

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R. Allikmets, N. Singh, H. Sun, N. F. Shroyer, A. Hutchinson, A. Chidambaram, B. Gerrard, L. Baird, D. Stauffer, A. Peiffer, A. Rattner, P. Smallwood, Y. Li, K. L. Anderson, R. A. Lewis, J. Nathans, M. Leppert, M. Dean, and J. R. Lupski, "A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy," Nat. Genet. 15, 236-246 (1997).
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T. S. Aleman, A. V. Cideciyan, E. A. M. Windsor, S. B. Schwartz, L. M. Gardner, J. M. Emmons, K. G. Duncan, J. D. Steinberg, E. M. Stone, and S. G. Jacobson, "Macular pigment in ABCA4-associated retinal degenerations: response to lutein supplementation," Invest. Ophthalmol. Visual Sci. 47, E-Abstract 5810 (2006).

S. G. Jacobson, A. V. Cideciyan, A. Sumaroka, T. S. Aleman, S. B. Schwartz, E. A. M. Windsor, A. J. Roman, E. M. Stone, and I. M. MacDonald, "Remodeling of the human retina in choroideremia--Rab Escort Protein 1 (REP-1) mutations," Invest. Ophthalmol. Visual Sci. 47, 4113-4120 (2006).
[CrossRef]

S. G. Jacobson, T. S. Aleman, A. V. Cideciyan, A. Sumaroka, S. B. Schwartz, E. A. M. Windsor, E. I. Traboulsi, E. Heon, S. J. Pittler, A. H. Milam, A. M. Maguire, K. Palczewski, E. M. Stone, and J. Bennett, "Identifying photoreceptors in blind eyes due to RPE65 mutations: prerequisite for human gene therapy success," Proc. Natl. Acad. Sci. U.S.A. 102, 6177-6182 (2005).
[CrossRef] [PubMed]

A. V. Cideciyan, M. Swider, T. S. Aleman, A. Sumaroka, S. B. Schwartz, M. I. Roman, A. H. Milam, J. Bennett, E. M. Stone, and S. G. Jacobson, "ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina," Invest. Ophthalmol. Visual Sci. 46, 4739-4746 (2005).
[CrossRef]

A. V. Cideciyan, T. S. Aleman, M. Swider, S. B. Schwartz, J. D. Steinberg, A. J. Brucker, A. M. Maguire, J. Bennett, E. M. Stone, and S. G. Jacobson, "Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence," Hum. Mol. Genet. 13, 525-534 (2004).
[CrossRef] [PubMed]

G. A. Fishman, E. M. Stone, D. A. Eliason, C. M. Taylor, M. Lindeman, and D. J. Derlacki, "ABCA4 gene sequence variations in patients with autosomal recessive cone-rod dystrophy," Arch. Ophthalmol. (Chicago) 121, 851-855 (2003).

A. R. Webster, E. Heon, A. J. Lotery, K. Vandenburgh, T. L. Casavant, K. T. Oh, G. Beck, G. A. Fishman, B. L. Lam, A. Levin, J. R. Heckenlively, S. G. Jacobson, R. G. Weleber, V. C. Sheffield, and E. M. Stone, "An analysis of allelic variation in the ABCA4 gene," Invest. Ophthalmol. Visual Sci. 42, 1179-1189 (2001).

G. A. Fishman, E. M. Stone, S. Grover, D. J. Derlacki, H. L. Haines, and R. R. Hockey, "Variation of clinical expression in patients with Stargardt dystrophy and sequence variations in the ABCR gene," Arch. Ophthalmol. (Chicago) 117, 504-510 (1999).

Stuck, B.

Sumaroka, A.

S. G. Jacobson, A. V. Cideciyan, A. Sumaroka, T. S. Aleman, S. B. Schwartz, E. A. M. Windsor, A. J. Roman, E. M. Stone, and I. M. MacDonald, "Remodeling of the human retina in choroideremia--Rab Escort Protein 1 (REP-1) mutations," Invest. Ophthalmol. Visual Sci. 47, 4113-4120 (2006).
[CrossRef]

S. G. Jacobson, T. S. Aleman, A. V. Cideciyan, A. Sumaroka, S. B. Schwartz, E. A. M. Windsor, E. I. Traboulsi, E. Heon, S. J. Pittler, A. H. Milam, A. M. Maguire, K. Palczewski, E. M. Stone, and J. Bennett, "Identifying photoreceptors in blind eyes due to RPE65 mutations: prerequisite for human gene therapy success," Proc. Natl. Acad. Sci. U.S.A. 102, 6177-6182 (2005).
[CrossRef] [PubMed]

A. V. Cideciyan, S. G. Jacobson, T. S. Aleman, D. Gu, S. E. Pearce-Kelling, A. Sumaroka, G. M. Acland, and G. D. Aguirre, "In vivo dynamics of retinal injury and repair in the rhodopsin mutant dog model of human retinitis pigmentosa," Proc. Natl. Acad. Sci. U.S.A. 102, 5233-5238 (2005).
[CrossRef] [PubMed]

A. V. Cideciyan, M. Swider, T. S. Aleman, A. Sumaroka, S. B. Schwartz, M. I. Roman, A. H. Milam, J. Bennett, E. M. Stone, and S. G. Jacobson, "ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina," Invest. Ophthalmol. Visual Sci. 46, 4739-4746 (2005).
[CrossRef]

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R. Allikmets, N. Singh, H. Sun, N. F. Shroyer, A. Hutchinson, A. Chidambaram, B. Gerrard, L. Baird, D. Stauffer, A. Peiffer, A. Rattner, P. Smallwood, Y. Li, K. L. Anderson, R. A. Lewis, J. Nathans, M. Leppert, M. Dean, and J. R. Lupski, "A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy," Nat. Genet. 15, 236-246 (1997).
[CrossRef] [PubMed]

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J. S. Sunness, M. D. Ziegler, and C. A. Applegate, "Issues in quantifying atrophic macular disease using retinal autofluorescence," Retina 26, 666-672 (2006).
[CrossRef] [PubMed]

Swider, M.

A. V. Cideciyan, M. Swider, T. S. Aleman, A. Sumaroka, S. B. Schwartz, M. I. Roman, A. H. Milam, J. Bennett, E. M. Stone, and S. G. Jacobson, "ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina," Invest. Ophthalmol. Visual Sci. 46, 4739-4746 (2005).
[CrossRef]

A. V. Cideciyan, T. S. Aleman, M. Swider, S. B. Schwartz, J. D. Steinberg, A. J. Brucker, A. M. Maguire, J. Bennett, E. M. Stone, and S. G. Jacobson, "Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence," Hum. Mol. Genet. 13, 525-534 (2004).
[CrossRef] [PubMed]

Taylor, C. M.

G. A. Fishman, E. M. Stone, D. A. Eliason, C. M. Taylor, M. Lindeman, and D. J. Derlacki, "ABCA4 gene sequence variations in patients with autosomal recessive cone-rod dystrophy," Arch. Ophthalmol. (Chicago) 121, 851-855 (2003).

Thompson, D. A.

P. A. Sieving, P. Chaudhry, M. Kondo, M. Provenzano, D. Wu, T. J. Carlson, R. A. Bush, and D. A. Thompson, "Inhibition of the visual cycle in vivo by 13-cis retinoic acid protects from light damage and provides a mechanism for night blindness in isotretinoin therapy," Proc. Natl. Acad. Sci. U.S.A. 98, 1835-1840 (2001).
[CrossRef] [PubMed]

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P. Kayatz, G. Thumann, T. T. Luther, J. F. Jordan, K. U. Bartz-Schmidt, P. J. Esser, and U. Schraermeyer, "Oxidation causes melanin fluorescence," Invest. Ophthalmol. Visual Sci. 42, 241-246 (2001).

Tijmes, N.

F. P. Cremers, D. J. van de Pol, M. van Driel, A. I. den Hollander, F. J. van Haren, N. V. Knoers, N. Tijmes, A. A. Bergen, K. Rohrschneider, A. Blankenagel, A. J. Pinckers, A. F. Deutman, and C. B. Hoyng, "Autosomal recessive retinitis pigmentosa and cone-rod dystrophy caused by splice site mutations in the Stargardt's disease gene ABCR," Hum. Mol. Genet. 7, 355-362 (1998).
[CrossRef] [PubMed]

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F. C. Piccolino, L. Borgia, E. Zinicola, M. Iester, and S. Torrielli, "Pre-injection fluorescence in indocyanine green angiography," Ophthalmology 103, 1837-1845 (1996).
[PubMed]

Traboulsi, E. I.

S. G. Jacobson, T. S. Aleman, A. V. Cideciyan, A. Sumaroka, S. B. Schwartz, E. A. M. Windsor, E. I. Traboulsi, E. Heon, S. J. Pittler, A. H. Milam, A. M. Maguire, K. Palczewski, E. M. Stone, and J. Bennett, "Identifying photoreceptors in blind eyes due to RPE65 mutations: prerequisite for human gene therapy success," Proc. Natl. Acad. Sci. U.S.A. 102, 6177-6182 (2005).
[CrossRef] [PubMed]

Travis, G. H.

G. H. Travis, M. Golczak, A. R. Moise, and K. Palczewski, "Diseases caused by defects in the visual cycle: retinoids as potential therapeutic agents," Annu. Rev. Pharmacol. Toxicol. 47, 8.1-8.44 (2007).
[CrossRef]

R. A. Radu, N. L. Mata, S. Nusinowitz, X. Liu, P. A. Sieving, and G. H. Travis, "Treatment with isotretinoin inhibits lipofuscin accumulation in a mouse model of recessive Stargardt's macular degeneration," Proc. Natl. Acad. Sci. U.S.A. 100, 4742-4747 (2003).
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Tucker, G. S.

G. S. Tucker, "Refractile bodies in the inner segments of cones in the aging human retina," Invest. Ophthalmol. Visual Sci. 27, 708-715 (1986).

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J. Weng, N. L. Mata, S. M. Azarian, R. T. Tzekov, D. G. Birch, and G. H. Travis, "Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice," Cell 98, 13-23 (1999).
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F. P. Cremers, D. J. van de Pol, M. van Driel, A. I. den Hollander, F. J. van Haren, N. V. Knoers, N. Tijmes, A. A. Bergen, K. Rohrschneider, A. Blankenagel, A. J. Pinckers, A. F. Deutman, and C. B. Hoyng, "Autosomal recessive retinitis pigmentosa and cone-rod dystrophy caused by splice site mutations in the Stargardt's disease gene ABCR," Hum. Mol. Genet. 7, 355-362 (1998).
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A. R. Webster, E. Heon, A. J. Lotery, K. Vandenburgh, T. L. Casavant, K. T. Oh, G. Beck, G. A. Fishman, B. L. Lam, A. Levin, J. R. Heckenlively, S. G. Jacobson, R. G. Weleber, V. C. Sheffield, and E. M. Stone, "An analysis of allelic variation in the ABCA4 gene," Invest. Ophthalmol. Visual Sci. 42, 1179-1189 (2001).

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A. Martinez-Mir, E. Paloma, R. Allikmets, C. Ayuso, T. del Rio, M. Dean, L. Vilageliu, R. Gonzalez-Duarte, and S. Balcells, "Retinitis pigmentosa caused by a homozygous mutation in the Stargardt disease gene ABCR," Nat. Genet. 18, 11-12 (1998).
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F. G. Holz, C. Bellman, S. Staudt, F. Schutt, and H. E. Volcker, "Fundus autofluorescence and development of geographic atrophy in age-related macular degeneration," Invest. Ophthalmol. Visual Sci. 42, 1051-1056 (2001).

F. G. Holz, C. Bellmann, M. Margaritidis, F. Schutt, T. P. Otto, and H. E. Volcker, "Patterns of increased in vivo fundus autofluorescence in the junctional zone of geographic atrophy of the retinal pigment epithelium associated with age-related macular degeneration," Graefe's Arch. Clin. Exp. Ophthalmol. 237, 145-152 (1999).
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C. Bellmann, F. G. Holz, O. Schapp, H. E. Volcker, and T. P. Otto, "[Topography of fundus autofluorescence with a new confocal scanning laser ophthalmoscope]," (in German) Ophthalmologe 94, 385-391 (1997).
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J. Hopkins, A. Walsh, and U. Chakravarthy, "Fundus autofluorescence in age-related macular degeneration: an epiphenomenon?" Invest. Ophthalmol. Visual Sci. 47, 2269-2271 (2006).
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C. Gerth, M. Andrassi-Darida, M. Bock, M. N. Preising, B. H. Weber, and B. Lorenz, "Phenotypes of 16 Stargardt macular dystrophy/fundus flavimaculatus patients with known ABCA4 mutations and evaluation of genotype-phenotype correlation," Graefes Arch. Clin. Exp. Ophthalmol. 240, 628-638 (2002).
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A. R. Webster, E. Heon, A. J. Lotery, K. Vandenburgh, T. L. Casavant, K. T. Oh, G. Beck, G. A. Fishman, B. L. Lam, A. Levin, J. R. Heckenlively, S. G. Jacobson, R. G. Weleber, V. C. Sheffield, and E. M. Stone, "An analysis of allelic variation in the ABCA4 gene," Invest. Ophthalmol. Visual Sci. 42, 1179-1189 (2001).

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A. W. Weinberger, A. Lappas, T. Kirschkamp, B. A. Mazinani, J. K. Huth, B. Mohammadi, and P. Walter, "Fundus near infrared fluorescence correlates with fundus near infrared reflectance," Invest. Ophthalmol. Visual Sci. 47, 3098-3108 (2006).
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F. C. Delori, C. K. Dorey, G. Staurenghi, O. Arend, D. G. Goger, and J. J. Weiter, "In vivo fluorescence of the ocular fundus exhibits retinal pigment epithelium lipofuscin characteristics," Invest. Ophthalmol. Visual Sci. 36, 718-729 (1995).

J. J. Weiter, F. C. Delori, G. L. Wing, and K. A. Fitch, "Retinal pigment epithelial lipofuscin and melanin and choroidal melanin in human eyes," Invest. Ophthalmol. Visual Sci. 27, 145-152 (1986).

Weleber, R. G.

G. A. Fishman, S. G. Jacobson, K. R. Alexander, A. V. Cideciyan, D. G. Birch, R. G. Weleber, and D. C. Hood, "Outcome measures and their application in clinical trials for retinal degenerative disease: outline, review, and perspective," Retina 25, 772-777 (2005).
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A. R. Webster, E. Heon, A. J. Lotery, K. Vandenburgh, T. L. Casavant, K. T. Oh, G. Beck, G. A. Fishman, B. L. Lam, A. Levin, J. R. Heckenlively, S. G. Jacobson, R. G. Weleber, V. C. Sheffield, and E. M. Stone, "An analysis of allelic variation in the ABCA4 gene," Invest. Ophthalmol. Visual Sci. 42, 1179-1189 (2001).

Weng, J.

J. Weng, N. L. Mata, S. M. Azarian, R. T. Tzekov, D. G. Birch, and G. H. Travis, "Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice," Cell 98, 13-23 (1999).
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M. Boulton, M. Rozanowska, B. Rozanowski, and T. Wess, "The photoreactivity of ocular lipofuscin," Photochem. Photobiol. Sci. 3, 759-764 (2004).
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D. Gibbs, S. M. Azarian, C. Lillo, J. Kitamoto, A. E. Klomp, K. P. Steel, R. T. Libby, and D. S. Williams, "Role of myosin VIIa and Rab27a in the motility and localization of RPE melanosomes," J. Cell. Sci. 117, 6473-6483 (2004).
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S. G. Jacobson, A. V. Cideciyan, A. Sumaroka, T. S. Aleman, S. B. Schwartz, E. A. M. Windsor, A. J. Roman, E. M. Stone, and I. M. MacDonald, "Remodeling of the human retina in choroideremia--Rab Escort Protein 1 (REP-1) mutations," Invest. Ophthalmol. Visual Sci. 47, 4113-4120 (2006).
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T. S. Aleman, A. V. Cideciyan, E. A. M. Windsor, S. B. Schwartz, L. M. Gardner, J. M. Emmons, K. G. Duncan, J. D. Steinberg, E. M. Stone, and S. G. Jacobson, "Macular pigment in ABCA4-associated retinal degenerations: response to lutein supplementation," Invest. Ophthalmol. Visual Sci. 47, E-Abstract 5810 (2006).

S. G. Jacobson, T. S. Aleman, A. V. Cideciyan, A. Sumaroka, S. B. Schwartz, E. A. M. Windsor, E. I. Traboulsi, E. Heon, S. J. Pittler, A. H. Milam, A. M. Maguire, K. Palczewski, E. M. Stone, and J. Bennett, "Identifying photoreceptors in blind eyes due to RPE65 mutations: prerequisite for human gene therapy success," Proc. Natl. Acad. Sci. U.S.A. 102, 6177-6182 (2005).
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Wing, G. L.

J. J. Weiter, F. C. Delori, G. L. Wing, and K. A. Fitch, "Retinal pigment epithelial lipofuscin and melanin and choroidal melanin in human eyes," Invest. Ophthalmol. Visual Sci. 27, 145-152 (1986).

Wolf, S.

S. Schmitz-Valckenberg, A. Bindewald-Wittich, J. Dolar-Szczasny, J. Dreyhaupt, S. Wolf, H. P. Scholl, F. G. Holz, and Fundus Autofluorescence in Age-Related Macular Degeneration Study Group, "Correlation between the area of increased autofluorescence surrounding geographic atrophy and disease progression in patients with AMD," Invest. Ophthalmol. Visual Sci. 47, 2648-2654 (2006).
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Wolffe, M.

Wu, D.

P. A. Sieving, P. Chaudhry, M. Kondo, M. Provenzano, D. Wu, T. J. Carlson, R. A. Bush, and D. A. Thompson, "Inhibition of the visual cycle in vivo by 13-cis retinoic acid protects from light damage and provides a mechanism for night blindness in isotretinoin therapy," Proc. Natl. Acad. Sci. U.S.A. 98, 1835-1840 (2001).
[CrossRef] [PubMed]

Ziegler, M. D.

J. S. Sunness, M. D. Ziegler, and C. A. Applegate, "Issues in quantifying atrophic macular disease using retinal autofluorescence," Retina 26, 666-672 (2006).
[CrossRef] [PubMed]

Zinicola, E.

F. C. Piccolino, L. Borgia, E. Zinicola, M. Iester, and S. Torrielli, "Pre-injection fluorescence in indocyanine green angiography," Ophthalmology 103, 1837-1845 (1996).
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Am. J. Ophthalmol. (1)

N. Lois, A. S. Halfyard, A. C. Bird, G. E. Holder, and F. W. Fitzke, "Fundus autofluorescence in Stargardt macular dystrophy--fundus flavimaculatus," Am. J. Ophthalmol. 138, 55-63 (2004).
[CrossRef] [PubMed]

Annu. Rev. Pharmacol. Toxicol. (1)

G. H. Travis, M. Golczak, A. R. Moise, and K. Palczewski, "Diseases caused by defects in the visual cycle: retinoids as potential therapeutic agents," Annu. Rev. Pharmacol. Toxicol. 47, 8.1-8.44 (2007).
[CrossRef]

Appl. Opt. (2)

Arch. Ophthalmol. (Chicago) (3)

G. A. Fishman, E. M. Stone, S. Grover, D. J. Derlacki, H. L. Haines, and R. R. Hockey, "Variation of clinical expression in patients with Stargardt dystrophy and sequence variations in the ABCR gene," Arch. Ophthalmol. (Chicago) 117, 504-510 (1999).

G. A. Fishman, E. M. Stone, D. A. Eliason, C. M. Taylor, M. Lindeman, and D. J. Derlacki, "ABCA4 gene sequence variations in patients with autosomal recessive cone-rod dystrophy," Arch. Ophthalmol. (Chicago) 121, 851-855 (2003).

N. Lois, G. E. Holder, C. Bunce, F. W. Fitzke, and A. C. Bird, "Phenotypic subtypes of Stargardt macular dystrophy--fundus flavimaculatus," Arch. Ophthalmol. (Chicago) 119, 359-369 (2001).

Biochemistry (1)

P. Maiti, J. Kong, S. R. Kim, J. R. Sparrow, R. Allikmets, and R. R. Rando, "Small molecule RPE65 antagonists limit the visual cycle and prevent lipofuscin formation," Biochemistry 45, 852-860 (2006).
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Br. J. Ophthamol. (2)

A. von Ruckmann, F. W. Fitzke, and A. C. Bird, "Distribution of fundus autofluorescence with a scanning laser ophthalmoscope," Br. J. Ophthamol. 79, 407-412 (1995).
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D. M. Paskowitz, M. M. Lavail, and J. L. Duncan, "Light and inherited retinal degeneration," Br. J. Ophthamol. 90, 1060-1066 (2006).
[CrossRef]

Cell (1)

J. Weng, N. L. Mata, S. M. Azarian, R. T. Tzekov, D. G. Birch, and G. H. Travis, "Insights into the function of Rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice," Cell 98, 13-23 (1999).
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Comput. Graph. Image Process. (1)

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Exp. Eye Res. (2)

G. E. Eldred and M. L. Katz, "Fluorophores of the human retinal pigment epithelium: separation and spectral characterization," Exp. Eye Res. 47, 71-86 (1988).
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J. R. Sparrow and M. Boulton, "RPE lipofuscin and its role in retinal pathobiology," Exp. Eye Res. 80, 595-606 (2005).
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Graefe's Arch. Clin. Exp. Ophthalmol. (1)

F. G. Holz, C. Bellmann, M. Margaritidis, F. Schutt, T. P. Otto, and H. E. Volcker, "Patterns of increased in vivo fundus autofluorescence in the junctional zone of geographic atrophy of the retinal pigment epithelium associated with age-related macular degeneration," Graefe's Arch. Clin. Exp. Ophthalmol. 237, 145-152 (1999).
[CrossRef]

Graefes Arch. Clin. Exp. Ophthalmol. (1)

C. Gerth, M. Andrassi-Darida, M. Bock, M. N. Preising, B. H. Weber, and B. Lorenz, "Phenotypes of 16 Stargardt macular dystrophy/fundus flavimaculatus patients with known ABCA4 mutations and evaluation of genotype-phenotype correlation," Graefes Arch. Clin. Exp. Ophthalmol. 240, 628-638 (2002).
[CrossRef] [PubMed]

Hum. Mol. Genet. (2)

A. V. Cideciyan, T. S. Aleman, M. Swider, S. B. Schwartz, J. D. Steinberg, A. J. Brucker, A. M. Maguire, J. Bennett, E. M. Stone, and S. G. Jacobson, "Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence," Hum. Mol. Genet. 13, 525-534 (2004).
[CrossRef] [PubMed]

F. P. Cremers, D. J. van de Pol, M. van Driel, A. I. den Hollander, F. J. van Haren, N. V. Knoers, N. Tijmes, A. A. Bergen, K. Rohrschneider, A. Blankenagel, A. J. Pinckers, A. F. Deutman, and C. B. Hoyng, "Autosomal recessive retinitis pigmentosa and cone-rod dystrophy caused by splice site mutations in the Stargardt's disease gene ABCR," Hum. Mol. Genet. 7, 355-362 (1998).
[CrossRef] [PubMed]

Invest. Ophthalmol. Visual Sci. (20)

J. J. Weiter, F. C. Delori, G. L. Wing, and K. A. Fitch, "Retinal pigment epithelial lipofuscin and melanin and choroidal melanin in human eyes," Invest. Ophthalmol. Visual Sci. 27, 145-152 (1986).

P. Kayatz, G. Thumann, T. T. Luther, J. F. Jordan, K. U. Bartz-Schmidt, P. J. Esser, and U. Schraermeyer, "Oxidation causes melanin fluorescence," Invest. Ophthalmol. Visual Sci. 42, 241-246 (2001).

G. S. Tucker, "Refractile bodies in the inner segments of cones in the aging human retina," Invest. Ophthalmol. Visual Sci. 27, 708-715 (1986).

M. Iwasaki and H. Inomata, "Lipofuscin granules in human photoreceptor cells," Invest. Ophthalmol. Visual Sci. 29, 671-679 (1988).

T. S. Aleman, A. V. Cideciyan, E. A. M. Windsor, S. B. Schwartz, L. M. Gardner, J. M. Emmons, K. G. Duncan, J. D. Steinberg, E. M. Stone, and S. G. Jacobson, "Macular pigment in ABCA4-associated retinal degenerations: response to lutein supplementation," Invest. Ophthalmol. Visual Sci. 47, E-Abstract 5810 (2006).

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Figures (4)

Fig. 1
Fig. 1

Illustration of different imaging modalities in a 43 - year -old representative normal subject. NIR-reflectance (a), SW-RAFI (b), and NIR-RAFI (c) mosaics of the central retina are shown spatially registered with respect to one another. Choroidal vasculature is apparent in some retinal regions with NIR imaging (arrows). Images are individually contrast stretched for visibility of features, and thus intensity of pixels is not comparable between images. Actual recorded RAFI intensities are shown (d) in gray-level units for SW-RAFI (gray trace) and NIR-RAFI (black trace) modalities along the horizontal meridian. An extended OCT mosaic along the horizontal meridian is also shown (e) coregistered to the profiles and the images. Retinal depth of zero corresponds to the approximate location of the RPE. Central macular region (single line) shows less backscatter signal originating from sub-RPE layers compared with more peripheral regions (double lines) where NIR light appears to penetrate deeper.

Fig. 2
Fig. 2

Interpretation of RAFI intensities in severe RPE disease in patient 7. (a) NIR-reflectance image showing a well-delineated macular lesion. The approximately two-fold greater reflectance within the lesion is likely due to depigmentation or atrophy of the RPE. (b) SW-RAFI of the central retina showing dramatically reduced lipofuscin autofluorescence within the lesion. Surrounding the lesion is an irregular annulus of hyperautofluorescence. (c) NIR-RAFI shows both similarities to and differences from SW-RAFI. Choroidal vessels are distinctly apparent within the lesion. Inset: OCT image obtained at an angle (dashed white line) through the fovea shows the transition from a high (triple lines) to a relatively low (single line) sub-RPE backscatter region at the temporal boundary of the lesion.

Fig. 3
Fig. 3

Qualitative and quantitative analyses of central disease between the fovea and the optic nerve head in a representative patient (patient 6) with early/mild A B C A 4 disease. (a) NIR-reflectance image showing the clinically overt maculopathy surrounded by normal appearing retina. (b) Microperimetry thresholds (in decibels, dB) with red-on-red stimulus. Stimuli not seen with red-on-red and white-on-red conditions are shown as x’s at the equivalent red-on-red threshold of 12.5 dB . (c) SW-RAFI image showing a parafoveal region of severe RPE disease. Intensity and run-length values quantified along the horizontal profile are shown on the right panels. (d) NIR-RAFI image shows similarities to and differences from SW-RAFI. Intensity and run-length values quantified along the horizontal profile are shown on the right panels. Regions of the retina with enrichment of choroidal component to the NIR-RAFI signal are marked as uninterpretable (x). All images (a,c,d) are shown individually contrast stretched for visibility of features. (e) OCT scan along the horizontal profile. Pair of overlaid white lines represents the normal limits of retinal thickness from the RPE peak (retinal depth of 0 μ m ). Estimated sub-RPE backscatter index (sRBI) is shown to the right. Boundaries of subclinical disease stages IIa and IIb are shown with vertical dashed lines coregistered in both columns across all panels. Pairs of gray lines on right panels represent normal limits ( mean ± 2 SD ) for each measure.

Fig. 4
Fig. 4

NIR-RAFI images and colocalized summary of all quantified parameters along the foveo–papillary profile in six patients. All images are shown individually contrast stretched for visibility of features. The parameters include microperimetry thresholds (MP/T); SW-RAFI intensity (SW-RAFI/I) and run-length (SW-RAFI/RL); NIR-RAFI intensity (NIR-RAFI/I) and run-length (NIR-RAFI/RL); and OCT-based sub-RPE backscattering index (OCT/sRBI). Parameters at each retinal location are classified into “within normal range” (graybars), “above normal range” (cross-hatched bars), and “below normal range” (hatched bars). Retinal loci where data were not available or NIR-RAFI was excluded because of above-normal sRBI values are specified as “not available/not used” (white). Boundaries of subclinical stage IIa and IIb disease are shown on NIR-RAFI images with dashed lines extended from parameter results.

Tables (1)

Tables Icon

Table 1 Clinical and Molecular Characteristics of Patients

Metrics