title: Retinitis pigmentosa: rod photoreceptor rescue by a calcium-channel blocker in the rd mouse.
abstract: Retinitis pigmentosa is an inherited degenerative disease of photoreceptors leading to blindness. A well-characterized model for this disease is provided by the retinal degeneration mouse, in which the gene for the rod cGMP phosphodiesterase is mutated, as in some affected human families. We report that D-cis-diltiazem, a calcium-channel blocker that also acts at light-sensitive cGMP-gated channels, rescued photoreceptors and preserved visual function in the retinal degeneration mouse. The long record of diltiazem prescription in cardiology should facilitate the design of clinical trials for some forms of retinitis pigmentosa.
authors.affiliation: Laboratoire de Physiopathologie Cellulaire et Moléculaire de la Rétine, Médicale A, BP 426, 1 place de l'hôpital, Université Louis Pasteur, 67091 Strasbourg, France.
authors.forename: M
authors.initials: M
authors.lastname: Frasson
authors.forename: J A
authors.initials: JA
authors.lastname: Sahel
authors.forename: M
authors.initials: M
authors.lastname: Fabre
authors.forename: M
authors.initials: M
authors.lastname: Simonutti
authors.forename: H
authors.initials: H
authors.lastname: Dreyfus
authors.forename: S
authors.initials: S
authors.lastname: Picaud
mesh_terms: D000818:Animals; D002121:Calcium Channel Blockers / Q000627:therapeutic use*; D006152:Cyclic GMP / Q000378:metabolism; D004110:Diltiazem / Q000627:therapeutic use*; D004195:Disease Models, Animal; D004596:Electroretinography; D015640:Ion Channel Gating; D051379:Mice; D008817:Mice, Mutant Strains; D018696:Neuroprotective Agents / Q000627:therapeutic use*; D010727:Phosphoric Diester Hydrolases / Q000235:genetics / Q000378:metabolism; D012160:Retina / Q000473:pathology; D017948:Retinal Rod Photoreceptor Cells / Q000187:drug effects*; D012174:Retinitis Pigmentosa / Q000188:drug therapy*
chemical_list: D002121:Calcium Channel Blockers; D018696:Neuroprotective Agents; D010727:Phosphoric Diester Hydrolases; D004110:Diltiazem; D006152:Cyclic GMP