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Crb1 is a determinant of retinal apical Müller glia cell features
Author(s) -
van de Pavert Serge A.,
Sanz Alicia Sanz,
Aartsen Wendy M.,
Vos Rogier M.,
Versteeg Inge,
Beck Susanne C.,
Klooster Jan,
Seeliger Mathias W.,
Wijnholds Jan
Publication year - 2007
Publication title -
glia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.954
H-Index - 164
eISSN - 1098-1136
pISSN - 0894-1491
DOI - 10.1002/glia.20561
Subject(s) - biology , retinal , retinal degeneration , retinitis pigmentosa , retina , retinal pigment epithelium , retinal regeneration , muller glia , microbiology and biotechnology , zebrafish , anatomy , genetics , neuroscience , gene , stem cell , progenitor cell , biochemistry
Mutations in the human Crumbs homologue‐1 ( CRB1 ) gene cause retinal blinding diseases, such as Leber congenital amaurosis and retinitis pigmentosa. In the previous studies we have shown that Crb1 resides in retinal Müller glia cells and that loss of Crb1 results in retinal degeneration (particularly in the inferior temporal quadrant of the mouse eye). Degeneration is increased by exposure to white light. Here, we studied the role of light and aging to gain a better understanding of the factors involved in the progress of retinal disease. Our data reveal that light is neither sufficient nor required to induce retinal disorganization and degeneration in young Crb1 −/− mutant mice, suggesting that it rather modulates the retinal phenotype. Gene expression profiling showed that expression of five genes is altered in light‐exposed Crb1 −/− mutant retinas. Three of the five genes are involved in chromosome stabilization (Pituitary tumor transforming gene 1 or Pttg1 , Establishment of cohesion 1 homolog 1 or Esco1 , and a gene similar to histone H2B ). In aged retinas, degeneration of photoreceptors, inner retinal neurons, and retinal pigment epithelium was practically limited to the inferior temporal quadrant. Loss of Crb1 in Müller glia cells resulted in an irregular number and size of their apical villi. We propose that Crb1 is required to regulate number and size of these Müller glia cell villi. The subsequent loss of retinal integrity resulted in neovascularization, in which blood vessels of the choroid protruded into the neural retina. © 2007 Wiley‐Liss, Inc.