Publication: Animal models and different therapies for treatment of retinitis pigmentosa
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Date
2009
Authors
Agurtzane Rivas, Miren ; Vecino, Elena
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Retinitis pigmentosa (RP) is a heterogeneous
group of retinal degenerative diseases initially affecting
the rod photoreceptor. Patients present with night
blindness, loss of peripheral vision and finally the loss of
central vision, as a consequence of death of cone
photoreceptors.
RP is a genetic disease, showing inheritance of
autosomal dominant (AD), autosomal recessive (AR) or
X-linked (XL) recessive traits, although some patients
have no family history of RP (simplex RP).
Many animal models of RP are available and have
led to a better understanding of the pathology of the
disease, and to the development of therapeutic strategies
aimed at curing or slowing down the genetic disorder.
In this review, we describe the selected animal
models (natural and transgenic) and their phenotypes
and genotypes, as well as the advantages and
disadvantages of the use of each animal. Also, we look
at different therapeutic strategies being studied
worldwide and report the latest results. Nevertheless,
many obstacles will have to be overcome before most of
these strategies can be applied to humans.
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