Inherited multifocal RPE-diseases:: mechanisms for local dysfunction in global retinoid cycle gene defects

被引：25

作者：

Besch, D ^{[1
]}

Jägle, H ^{[1
]}

Scholl, HPN ^{[1
]}

Seeliger, MW ^{[1
]}

Zrenner, E ^{[1
]}

机构：

[1] Univ Tubingen, Hosp Eye, D-72076 Tubingen, Germany

来源：

VISION RESEARCH | 2003年 / 43卷 / 28期

关键词：

visual cycle; gene; retinoid; retinal pigment epithelium; retinal disease;

D O I：

10.1016/j.visres.2003.09.020

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

Alterations of retinoid cycle genes are known to cause retinal diseases characterized by focal white dot fundus lesions. Fundus appearances reveal circumscribed RPE-changes, although generalized metabolic defects and global functional abnormalities are present. As a possible explanation, topographic inhomogeneities of the human photoreceptor mosaic and the role of a cone specific visual cycle will be discussed. Due to particular characteristics of photoreceptor subtypes as well as different pathways for photopigment regeneration the metabolic demand of individual RPE cells might differ. In "flecked retina diseases" heterogeneity of metabolic demand in individual RPE cells could therefore be responsible for their multifocal appearance. (C) 2003 Elsevier Ltd. All rights reserved.

引用

页码：3095 / 3108

页数：14

共 98 条

[1] EFFECT OF LIGHT ON ENDOGENOUS LIGANDS CARRIED BY INTERPHOTORECEPTOR RETINOID-BINDING PROTEIN [J].