Animal modelling for inherited central vision loss.

Détails

Ressource 1Télécharger: BIB_55559404111F.P001.pdf (976.64 [Ko])
Etat: Serval
Version: Final published version
ID Serval
serval:BIB_55559404111F
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
Animal modelling for inherited central vision loss.
Périodique
Journal of Pathology
Auteur(s)
Kostic C., Arsenijevic Y.
ISSN
1096-9896 (Electronic)
ISSN-L
0022-3417
Statut éditorial
Publié
Date de publication
2016
Peer-reviewed
Oui
Volume
238
Numéro
2
Pages
300-310
Langue
anglais
Notes
Publication types: Journal Article ; Review
Publication Status: ppublish
Résumé
Disease-causing variants of a large number of genes trigger inherited retinal degeneration leading to photoreceptor loss. Because cones are essential for daylight and central vision such as reading, mobility, and face recognition, this review focuses on a variety of animal models for cone diseases. The pertinence of using these models to reveal genotype/phenotype correlations and to evaluate new therapeutic strategies is discussed. Interestingly, several large animal models recapitulate human diseases and can serve as a strong base from which to study the biology of disease and to assess the scale-up of new therapies. Examples of innovative approaches will be presented such as lentiviral-based transgenesis in pigs and adeno-associated virus (AAV)-gene transfer into the monkey eye to investigate the neural circuitry plasticity of the visual system. The models reported herein permit the exploration of common mechanisms that exist between different species and the identification and highlighting of pathways that may be specific to primates, including humans.
Mots-clé
Animals, Animals, Genetically Modified, Blindness/genetics, Blindness/therapy, Disease Models, Animal, Dogs, Genetic Therapy, Humans, Mutation/genetics, Retinal Cone Photoreceptor Cells/physiology, Retinal Degeneration/genetics, Retinal Degeneration/therapy, Retinitis Pigmentosa/genetics, Retinitis Pigmentosa/therapy, Rodentia, Saimiri, Sheep, Swine
Pubmed
Web of science
Open Access
Oui
Création de la notice
05/10/2015 11:55
Dernière modification de la notice
08/05/2019 18:47
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