scholarly journals Rasagiline delays retinal degeneration in a mouse model of retinitis pigmentosa via modulation of Bax/Bcl-2 expression

2018 ◽  
Vol 24 (5) ◽  
pp. 448-455 ◽  
Author(s):  
Ana B. Garcia-Delgado ◽  
Lourdes Valdés-Sánchez ◽  
Sofia M. Calado ◽  
Francisco J. Diaz-Corrales ◽  
Shom S. Bhattacharya
Keyword(s):  
Mouse Model ◽  
Retinitis Pigmentosa ◽  
Retinal Degeneration

HIF‐1α stabilization reduces retinal degeneration in a mouse model of retinitis pigmentosa

2018 ◽  
Vol 32 (5) ◽  
pp. 2438-2451 ◽  
Author(s):  
Lorena Olivares‐Gonza´lez ◽  
Cristina Martínez‐Fernandez de la Ca´mara ◽  
David Herva´s ◽  
Jose´ Mar´a Milla´n ◽  
Regina Rodrigo
Keyword(s):  
Mouse Model ◽  
Retinitis Pigmentosa ◽  
Retinal Degeneration

scholarly journals Study of Protein Targeting and Mislocalization in Rod Photoreceptors

2021 ◽  
Vol 4 (1) ◽  
Author(s):  
Asael Nunez ◽  
Shimpei Takita ◽  
Sanae Imanishi ◽  
Yoshikazu Imanishi
Keyword(s):  
Plasma Membrane ◽  
Mouse Model ◽  
Retinitis Pigmentosa ◽  
Retinal Degeneration ◽  
Molecular Mechanisms ◽  
Major Protein ◽  
Membrane Expression ◽  
Rich Region ◽  
Rod Photoreceptors ◽  
Proline Rich Region

The photoreceptor outer segment (OS) is a highly specialized organelle for light absorption. Precise localization of OS resident proteins is important for photoreceptor function. Molecular mechanisms underlying OS targeting of proteins and their mislocalization, which frequently causes inherited retinal degeneration, have been intensely investigated. Rhodopsin, a major protein of the rod OS, is often mislocalized to the inner segment (IS) plasma membrane of rod photoreceptors in retinal degeneration patients. In the Xenopus laevis model of retinitis pigmentosa, we previously found that Na+/K+-ATPase (NKA), a major IS protein, was downregulated. The Imanishi lab recently created a novel retinitis pigmentosa mouse model carrying the Q344ter rhodopsin gene mutation, which causes rhodopsin mislocalization to the rod IS plasma membrane. In this summer program, we examined whether this mouse model also displays reduced NKA expression in the rod IS’s by immunohistochemistry at postnatal day 30. Although NKA was properly localized to the IS plasma membrane, expression of NKA was reduced in mutant photoreceptors compared to wildtype cells. In the rod OS, activation of rhodopsin eventually leads to the closure of the cyclic nucleotide gated (CNG) channel, which consists of a and b subunits. This channel localizes to the OS plasma membrane, and the N-terminal proline-rich region (R) of the b subunit (CNGb1) may be important for its interaction with peripherin (PRPH2), another OS resident protein. Currently, it is not well understood whether this interaction is necessary for the proper localization of CNGb1 to the OS plasma membrane. Using Xenopus as a model, we studied the role of the N-terminal proline-rich region in properly localizing CNGb1 to the OS plasma membrane by generating transgenic CNGb1(DR) tadpoles that expressed CNGb1(DR) in rods under the control of a rhodopsin promoter. We found that CNGb1(DR) properly localized to the OS plasma membrane. 

scholarly journals Author response: Small molecule Photoregulin3 prevents retinal degeneration in the RhoP23H mouse model of retinitis pigmentosa

2017 ◽  
Author(s):  
Paul A Nakamura ◽  
Andy A Shimchuk ◽  
Shibing Tang ◽  
Zhizhi Wang ◽  
Kole DeGolier ◽  
...  
Keyword(s):  
Mouse Model ◽  
Retinitis Pigmentosa ◽  
Retinal Degeneration ◽  
Small Molecule ◽  
Author Response

Zinc–desferrioxamine attenuates retinal degeneration in the rd10 mouse model of retinitis pigmentosa

2011 ◽  
Vol 51 (8) ◽  
pp. 1482-1491 ◽  
Author(s):  
Alexey Obolensky ◽  
Eduard Berenshtein ◽  
Michal Lederman ◽  
Baruch Bulvik ◽  
Ruslana Alper-Pinus ◽  
...  
Keyword(s):  
Mouse Model ◽  
Retinitis Pigmentosa ◽  
Retinal Degeneration
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