Loss of the Cholesterol-binding Protein Prominin-1/CD133 Causes Disk Dysmorphogenesis and Photoreceptor Degeneration

Overview

Journal J Neurosci

Specialty Neurology

Date 2009 Feb 21

PMID 19228982

Citations 100

Authors

Serena Zacchigna

Hideyasu Oh

Michaela Wilsch-Brauninger

Ewa Missol-Kolka

Jozsef Jaszai

Sandra Jansen

Naoyuki Tanimoto

Felix Tonagel

Mathias Seeliger

Wieland B Huttner

Denis Corbeil

Mieke Dewerchin

Stefan Vinckier

Lieve Moons

Peter Carmeliet

Affiliations

Soon will be listed here.

Abstract

Prominin-1/CD133 (Prom-1) is a commonly used marker of neuronal, vascular, hematopoietic and other stem cells, yet little is known about its biological role and importance in vivo. Here, we show that loss of Prom-1 results in progressive degeneration of mature photoreceptors with complete loss of vision. Despite the expression of Prom-1 on endothelial progenitors, photoreceptor degeneration was not attributable to retinal vessel defects, but caused by intrinsic photoreceptor defects in disk formation, outer segment morphogenesis, and associated with visual pigment sorting and phototransduction abnormalities. These findings shed novel insight on how Prom-1 regulates neural retinal development and phototransduction in vertebrates.

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