Literature DB >> 15180268

Does constitutive phosphorylation protect against photoreceptor degeneration in Rpe65-/- mice?

Baerbel Rohrer1, Zsolt Ablonczy, Sergei Znoiko, Michael Redmond, Jian-Xing Ma, Rosalie Crouch.   

Abstract

Despite the presence of this virgin opsin, Rpe65-/- rods are behaving like dark-adapted rods. These results argue that opsin which has not been exposed to 11-cis retinal and is constitutively phosphorylated, does not generate the activity generally associated with the bleached apoprotein. However, increased light-independent activation of transducin (due to bleached opsin) could be demonstrated after the addition of exogenous 11-cis retinal. We hypothesize that free opsin in the Rpe65-/- rods does not cause degeneration of rods by constitutive activation of the phototransduction cascade; but rather rods may die due to other causes such as the impairment of RPE function due to excess unprocessed retinyl-esters in the RPE.

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Year:  2003        PMID: 15180268     DOI: 10.1007/978-1-4615-0067-4_28

Source DB:  PubMed          Journal:  Adv Exp Med Biol        ISSN: 0065-2598            Impact factor:   2.622


  1 in total

1.  Gene therapy rescues cone structure and function in the 3-month-old rd12 mouse: a model for midcourse RPE65 leber congenital amaurosis.

Authors:  Xia Li; Wensheng Li; Xufeng Dai; Fansheng Kong; Qinxiang Zheng; Xiangtian Zhou; Fan Lü; Bo Chang; Bärbel Rohrer; William W Hauswirth; Jia Qu; Ji-jing Pang
Journal:  Invest Ophthalmol Vis Sci       Date:  2011-01-05       Impact factor: 4.799

  1 in total

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