Characterization of rhodopsin mis-sorting and constitutive activation in a transgenic rat model of retinitis pigmentosa

Investigative Ophthalmology and Visual Science

Volumn 41, Issue 6, 2000, Pages 1546-1553

  Green, Eric S ^a   Menz, Michael D ^a   LaVail, Matthew M ^b   Flannery, John G ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

COMPLEMENTARY DNA; MESSENGER RNA; RHODOPSIN;

ANIMAL CELL; ANIMAL MODEL; ARTICLE; CELL DEATH; ELECTRORETINOGRAPHY; EXPERIMENTAL MODEL; MALE; NONHUMAN; PRIORITY JOURNAL; RAT; RETINA DEGENERATION; RETINITIS PIGMENTOSA; TRANSGENIC MOUSE;

ANIMALS; ANIMALS, GENETICALLY MODIFIED; APOPTOSIS; BIOLOGICAL TRANSPORT; BLOTTING, WESTERN; DISEASE MODELS, ANIMAL; ELECTRORETINOGRAPHY; FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT; PHOTOTRANSDUCTION; POINT MUTATION; RATS; RATS, SPRAGUE-DAWLEY; RETINITIS PIGMENTOSA; RHODOPSIN; RODS (RETINA);

EID: 0034015064     PISSN: 01460404     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (33)

1. Sakmar TP. Rhodopsin: a prototypical G protein-coupled receptor. Prog Nucleic Acid Res Mol Biol. 1998;59:1-34
2. Sung CH, Makino C, Baylor D, Nathans J. A rhodopsin gene mutation responsible for autosomal dominant retinitis pigmentosa results in a protein that is defective in localization to the photoreceptor outer segment. J Neurosci. 1994;14:5818-5833.
3. Li T, Snyder WK, Olsson JE, Dryja TP. Transgenic mice carrying the dominant rhodopsin mutation P347S: evidence for defective vectorial transport of rhodopsin to the outer segments. Proc Natl Acad Sci USA. 1996;93:14176-14181.
4. Hurley JB, Spencer M, Niemi GA. Rhodopsin phosphorylation and its role in photoreceptor function. Vision Res. 1998;38:1341-1352.
5. Chen J, Makino CL, Peachey NS, Baylor DA, Simon MI. Mechanisms of rhodopsin inactivation in vivo as revealed hy a COOH-terminal truncation mutant. Science. 1995;267:374-377.
6. Travis GH. Mechanisms of cell death in the inherited retinal degenerations. Am J Hum Genet. 1998;62:503-508.
7. Remé CE, Grimm C, Hafezi F, Marti A, Wenzel A. Apoptotic cell death in retinal degenerations. Prog Retin Eye Res. 1998;17:443-464.
8. Tso MO, Zhang C, Abler AS, et al. Apoptosis leads to photoreceptor degeneration in inherited retinal dystrophy of RCS rats. Invest Ophthalmol Vis Sci. 1994;35:2693-2699.
9. Chang GQ, Hao Y, Wong F. Apoptosis: final common pathway of photoreceptor death in rd, rds, and rhodopsin mutant mice. Neuron. 1993;11:595-605.
10. Rao VR, Oprian DD. Activating mutations of rhodopsin and other G protein-coupled receptors. Annu Rev Biophys Biomol Struct. 1996;25:287-314.
11. Sieving PA, Richards JE, Naarendorp F, et al. Dark light: model for nightblindness from the human rhodopsin Gly-90->Asp mutation. Proc Natl Acad Sci USA. 1995;92:880-884.
12. Robinson PR, Buczylko J, Ohguro H, Palczewski K. Opsins with mutations at the site of chromophore attachment constitutively activate transducin but are not phosphorylated by rhodopsin kinase. Proc Natl Acad Sci USA. 1994;91:5411-5415.
13. Olsson JE, Gordon JW, Pawlyk BS, et al. Transgenic mice with a rhodopsin mutation (Pro23His): a mouse model of autosomal dominant retinitis pigmentosa. Neuron. 1992;9:815-830.
14. Steinberg RH, Flannery JG, Naash M, et al. Transgenic rat models of inherited retinal degeneration caused by mutant opsin genes [ARVO Abstract]. Invest Ophthalmol Vis Sci. 1996;37(3):S698. Abstract nr 3190.
15. Newman GR, Hobot JA, eds. Resin Microscopy and On-Section Immunocytochemistry. Berlin: Springer-Verlag: 1993.
16. McDonald K, Morphew MK. Improved preservation of ultrastructure in difficult-to-fix organisms by high pressure freezing and freeze substitution, I: Drosophila melanogaster and Strongylocentrotus purpuratus embryos. Microsc Res Tech. 1993;24:465-473.
17. Lewin AS, Drenser KA, Hauswirth WW, et al. Ribozyme rescue of photoreceptor cells in a transgenic rat model of autosomal dominant retinitis pigmentosa. Nat Med. 1998;4:967-971.
18. Morrow EM, Belliveau MJ, Cepko CL. Two phases of rod photoreceptor differentiation during rat retinal development. J Neurosci. 1998;18:3738-3748.
19. Goto Y. An electrode to record the mouse cornea electroretinogram. Doc Ophthalmol. 1996;91:147-154.
20. Michon JJ, Li ZL, Shioura N, Anderson RJ, Tso MO. A comparative study of methods of photoreceptor morphometry. Invest Ophthalmol Vis Sci. 1991;32:280-284.
21. Deretic D, Schmerl S, Hargrave PA, Arendt A, McDowell JH. Regulation of sorting and post-Golgi trafficking of rhodopsin by its C-terminal sequence QVS(A)PA. Proc Natl Acad Sci USA. 1998;95: 10620-10625.
22. Chuang JZ, Sung CH. The cytoplasmic tail of rhodopsin acts as a novel apical sorting signal in polarized MDCK cells. J Cell Biol. 1998;142:1245-1256.
23. Roof DJ, Adamian M, Haves A. Rhodopsin accumulation at abnormal sites in retinas of mice with a human P23H rhodopsin transgene. Invest Ophthalmol Vis Sci. 1994;35:4049-4062.
24. Liu X, Wu TH, Stowe S, et al. Defective phototransductive disk membrane morphogenesis in transgenic mice expressing opsin with a mutated N-terminal domain. J Cell Sci. 1997;110:2589-2597.
25. LaVail MM. Kinetics of rod outer segment renewal in the developing mouse retina. J Cell Biol. 1973;58:650-661.
26. Treisman JE, Morabito MA, Barnstable CJ. Opsin expression in the rat retina is developmentally regulated by transcriptional activation. Mol Cell Biol. 1988;8:1570-1579.
27. Kurada P, O'Tousa JE. Retinal degeneration caused by dominant rhodopsin mutations in Drosophila. Neuron. 1995;14:571-579.
28. Colley NJ, Cassill JA, Baker EK, Zuker CS. Defective intracellular transport is the molecular basis of rhodopsin-dependent dominant retinal degeneration. Proc Natl Acad Sci USA. 1995;92:3070-3074.
29. Battelle BA, LaVail MM. Rhodopsin content and rod outer segment length in albino rat eyes: modification by dark adaptation. Exp Eye Res. 1978;26:487-497.
30. Dryja TP, Finn JT, Peng YW, et al. Mutations in the gene encoding the alpha subunit of the rod cGMP-gated channel in autosomal recessive retinitis pigmentosa. Proc Natl Acad Sci USA. 1995;92: 10177-10181.
31. Lisman J, Fain G. Support for the equivalent light hypothesis for RP. Nat Med. 1995;1:1254-1255.
32. Li T, Franson WK, Gordon JW, Berson EL, Dryja TP. Constitutive activation of phototransduction by K296E opsin is not a cause of photoreceptor degeneration. Proc Natl Acad Sci USA. 1995;92: 3551-3555.
33. Keen TJ, Inglehearn CF, Lester DH, et al. Autosomal dominant retinitis pigmentosa: four new mutations in rhodopsin, one of them in the retinal attachment site. Genomics. 1991;11:199-205.