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Titolo:
Calcium channel blocker D-cis-diltiazem does not slow retinal degenerationin the PDE6B mutant rcd1 canine model of retinitis pigmentosa
Autore:
Pearce-Kelling, SE; Aleman, TS; Nickle, A; Laties, AM; Aguirre, GD; Jacobson, SG; Acland, GM;
Indirizzi:
Univ Penn, Scheie Eye Inst, Dept Ophthalmol, Philadelphia, PA 19104 USA Univ Penn Philadelphia PA USA 19104 phthalmol, Philadelphia, PA 19104 USA Cornell Univ, Coll Vet Med, Baker Inst, Ithaca, NY 14853 USA Cornell UnivIthaca NY USA 14853 et Med, Baker Inst, Ithaca, NY 14853 USA Univ Penn, Retinal Dis Studies Facil, Kennett Square, PA USA Univ Penn Kennett Square PA USA is Studies Facil, Kennett Square, PA USA
Titolo Testata:
MOLECULAR VISION
fascicolo: 7, volume: 7, anno: 2001,
pagine: 42 - 47
SICI:
1090-0535(20010228)7:7<42:CCBDDN>2.0.ZU;2-I
Fonte:
ISI
Lingua:
ENG
Soggetto:
ROD-CONE DYSPLASIA; BETA-SUBUNIT GENE; CGMP-PHOSPHODIESTERASE; PHOTORECEPTOR CELLS; IRISH SETTERS; RD MOUSE; NONSENSE MUTATION; PROCTOR-LECTURE; DOG; DEFECT;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
39
Recensione:
Indirizzi per estratti:
Indirizzo: Jacobson, SG Univ Penn, Scheie Eye Inst, Dept Ophthalmol, 51 N 39th St, Philadelphia, PA 19104 USA Univ Penn 51 N 39th St Philadelphia PA USA 19104 PA 19104 USA
Citazione:
S.E. Pearce-Kelling et al., "Calcium channel blocker D-cis-diltiazem does not slow retinal degenerationin the PDE6B mutant rcd1 canine model of retinitis pigmentosa", MOL VIS, 7(7), 2001, pp. 42-47

Abstract

Purpose: D-cis-diltiazem, a calcium channel blocker, has been reported to enhance photoreceptor survival in the rd mouse, a model of retinitis pigmentosa (RP) resulting from mutation of the PDE6B gene. We tested the hypothesis that diltiazem treatment would similarly rescue the canine rcd1 model ofRP, which is also caused by a null mutation in the PDE6B gene. Methods: D-cis-diltiazem was delivered orally twice daily to rcd1 affecteddogs beginning at 4 weeks of age; untreated age-matched rcd1 dogs served as controls. At 14 weeks, electroretinograms (ERG) were performed on all animals; 14 dogs were euthanized at this age, and 2 dogs at 25 weeks of age. Eyes were enucleated, fixed, and processed for routine histological examination. Results: No significant differences were found in ERG or histopathologic parameters between diltiazem-treated and untreated rcd1 dogs. Neither rcd1 group showed a rod b-wave; ERGs evoked by single white flashes (dark- or light-adapted) and flicker were also identical between groups. Similarly, treated and untreated animals did not differ in the degree of preservation of the photoreceptor layer, confirmed in cell counts within the outer nuclear layer. Conclusions: Treatment of rcd1 affected dogs with D-cis-diltiazem did not modify the photoreceptor disease when results were assessed using either ERG or histopathologic criteria. The positive photoreceptor-rescue effect of calcium channel blockers reported in the rd mouse was thus not generalizable to another species with retinal degeneration due to mutation in the PDE6Bgene. Caution needs to be exerted in extrapolation to the comparable humanforms of RP.

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Documento generato il 31/03/20 alle ore 19:16:13