Delayed dark-adaptation and lipofuscin accumulation in abcr+/- mice: Implications for involvement of ABCR in age-related macular degeneration

Investigative Ophthalmology and Visual Science

Volumn 42, Issue 8, 2001, Pages 1685-1690

  Mata, N L ^a   Tzekov, R T ^a   Liu, X ^a   Weng, J ^a   Birch, D G ^a   Travis, G H ^a  

^a JULES STEIN EYE INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

11 CIS RETINAL; ABC TRANSPORTER; ALDEHYDE DERIVATIVE; DIHYDRO N RETINYLIDENE N RETINYLPHOSPHATIDYLETHANOLAMINE; LIPOFUSCIN; N RETINYLIDENE N RETINYLETHANOLAMINE; N RETINYLIDENE N RETINYLPHOSPHATIDYLETHANOLAMINE; PHOSPHOLIPID; RETINAL; RETINOID; UNCLASSIFIED DRUG;

AGE; AGING; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BLEACHING; CONTROLLED STUDY; DARK ADAPTATION; ELECTRON MICROSCOPY; ELECTRORETINOGRAPHY; GENETIC PREDISPOSITION; HETEROZYGOTE; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; LIGHT EXPOSURE; MICROSCOPY; MOUSE; NONHUMAN; PHENOTYPE; PIGMENT EPITHELIUM; PRIORITY JOURNAL; RECESSIVE GENE; RETINA MACULA DEGENERATION; RETINA ROD; STARGARDT DISEASE; TISSUE SECTION;

ANIMALS; ATP-BINDING CASSETTE TRANSPORTERS; CHROMATOGRAPHY, HIGH PRESSURE LIQUID; DARK ADAPTATION; ELECTRORETINOGRAPHY; LIPOFUSCIN; MACULAR DEGENERATION; MICE; MICE, MUTANT STRAINS; PHENOTYPE; PIGMENT EPITHELIUM OF EYE; RETINOIDS; ROD OUTER SEGMENTS;

EID: 0034944895     PISSN: 01460404     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (45)

1. A photoreceptor cell-specific ATP-binding transporter gene (ABCR) is mutated in recessive Stargardt macular dystrophy
2. Allelic variation in ABCR associated with Stargardt disease but not age-related macular degeneration
3. Delayed rod dark adaptation in patients with Stargardt's disease
4. Histopathology and immunocytochemistry of the neurosensory retina in fundus flavimaculatus
5. Cell loss in the aging retina. Relationship to lipofuscin accumulation and macular degeneration
6. Symptomatic abnormalities of dark adaptation in patients with age-related Bruch's membrane change
7. Morphologic changes in age-related maculopathy
8. Macular function impairment in eyes with early age-related macular degeneration
9. Autofluorescence distribution associated with drusen in age-related macular degeneration
10. Mutation of the Stargardt disease gene (ABCR) in age-related macular degeneration
11. New ABCR mutations and clinical phenotype in Italian patients with Stargardt disease
12. Further evidence for an association of ABCR alleles with age-related macular degeneration
13. Analysis of the Stargardt disease gene (ABCR) in age-related macular degeneration
14. ABCR gene analysis in familial exudative age-related macular degeneration
15. Immunocytochemical localization of a large intrinsic membrane protein to the incisures and margins of frog rod outer segment disks
16. The photoreceptor Rim protein is an ABC transporter encoded by the gene for recessive Stargardts-disease (ABCR)
17. The 220-kDa rim protein of retinal rod outer segments is a member of the ABC transporter superfamily
18. Retinal stimulates ATP hydrolysis by purified and reconstituted ABCR, the photoreceptor-specific ATP-binding cassette transporter responsible for Stargardt disease
19. Insights into the function of rim protein in photoreceptors and etiology of Stargardt's disease from the phenotype in abcr knockout mice
20. The effect of lipid environment and retinoids on the ATPase activity of ABCR, the photoreceptor transporter responsible for Stargardt macular dystrophy
21. Lipofuscin of the retinal pigment epithelium: A review
22. Ocular Age Pigment "A2-E": An unprecedented pyridinium bisretinoid
23. Lipofuscin in the retina: Quantitative assay for an unprecedented autofluorescent compound (pyridinium bis-retinoid, A2-E) of ocular age pigment
24. Biosynthesis of a major lipofuscin fluorophore in mice and humans with ABCR-mediated retinal and macular degeneration
25. A quantitative account of the activation steps involved in phototransduction in amphibian photoreceptors
26. 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
27. Psychophysical evidence for rod vulnerability in age-related macular degeneration
28. Transducin activation by the bovine opsin apoprotein
29. Opsin/all-trans-retinal complex activates transducin by different mechanisms than photolyzed rhodopsin
30. A comparison of the efficiency of G protein activation by ligand-free and light-activated forms of rhodopsin
31. Diffusible ligand all-trans-retinal activates opsin via a palmitoylation-dependent mechanism
32. The biosynthesis of A2E, a fluorophore of aging retina, involves the formation of the precursor, A2-PE, in the photoreceptor outer segment membrane
33. Lipofuscin accumulation in cultured retinal pigment epithelial cells reduces their phagocytic capacity
34. Inhibition of lysosomal degradative functions in RPE cells by a retinoid component of lipofuscin
35. Retinal age pigments generated by self-assembling lysosomotropic detergents
36. A2E, a lipofuscin fluorophore, in human retinal pigmented epithelial cells in culture
37. Photodamage to human RPE cells by A2-E, a retinoid component of lipofuscin
38. Count and density of human retinal photoreceptors
39. The topography and age relationship of lipofuscin concentration in the retinal pigment epithelium
40. Retinitis pigmentosa caused by a homozygous mutation in the Stargardt-disease gene ABCR
41. Genotype/phenotype analysis of a photoreceptor-specific ATP-binding cassette transporter gene, ABCR, in Stargardt disease
42. Mutations in the ABCA4 (ABCR) gene are the major cause of autosomal recessive cone-rod dystrophy
43. The relationships of age changes in retinal pigment epithelium and Bruch's membrane
44. The ultrastructure of drusen
45. Clinico-morphologic correlations of drusen of Bruch's membrane