Mechanisms of apoptosis in retinitis pigmentosa.

Détails

ID Serval
serval:BIB_940CD165EADC
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Mechanisms of apoptosis in retinitis pigmentosa.
Périodique
Current molecular medicine
Auteur(s)
Cottet S., Schorderet D.F.
ISSN
1566-5240
Statut éditorial
Publié
Date de publication
2009
Peer-reviewed
Oui
Volume
9
Numéro
3
Pages
375-83
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't ; Review - Publication Status: ppublish
Résumé
Mutations in humans are associated with several forms of inherited retinal dystrophies, such as Retinitis Pigmentosa which lead to retinal cell death and irreversible loss of vision. Genes involved in affected patients mainly encode proteins related to vision physiology including visual cycle and light-dependent phototransduction cascade. As reported in spontaneous and genetically engineered mouse models, apoptosis is a common fate in retinal degeneration, although the triggered signals to retinal apoptosis remain largely unraveled. Several studies highlighted that many of the molecular pathways involved in ocular diseases rely on caspase-dependent or -independent apoptotic mitochondrial pathway involving the Bcl-2 family of proteins. Anti- and pro-apoptotic Bcl-2 members are present in retinal tissues and are thought to play a role in the pathogenesis of several retinal disorders. Since almost no efficient treatments are available so far, it remains a great challenge to decipher the molecular pathways involved in retinal dystrophies and to develop alternative therapies to prevent or inhibit eye defect. Toward this goal, mutation-independent strategies such as molecular therapy provides promising and exciting approaches to deliver anti-apoptotic molecules targeting the Bcl-2 pathway through the use of cell permeable transport peptides. Modulation of common apoptotic signaling pathways may be of outstanding potential to target multiple retinal dystrophies regardless of the primary genetic defect.
Mots-clé
Animals, Apoptosis/physiology, Caspases/metabolism, Humans, Light Signal Transduction/physiology, Mutation, Retinitis Pigmentosa/genetics, Retinitis Pigmentosa/pathology, Signal Transduction/physiology
Pubmed
Web of science
Création de la notice
30/09/2009 17:15
Dernière modification de la notice
20/03/2018 13:18
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