Article
Photoreceptor-induced RPE phagolysosomal maturation defects in Stargardt-like Maculopathy (STGD3).
Scientific reports - 13 Apr 2018
Dejos Camille, Kuny Sharee, Han Woo Hyun, Capel Heather, Lemieux Hélène, Sauvé Yves
Abstract excerpt
For many neurodegenerative disorders, expression of a pathological protein by one cell type impedes function of other cell types, which in turn contributes to the death of the first cell type. In transgenic mice modelling Stargardt-like (STGD3) maculopathy, human mutant ELOVL4 expression by photoreceptors is associated with defects in the underlying retinal pigment epithelium (RPE). To examine how photoreceptors...
Topics
- Animals
- Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
- Cathepsin D
- Cell Line
- Disease Models, Animal
- Eye Proteins
- Humans
- Lysosomes
- Macular Degeneration
- Membrane Proteins
- Mice