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http://purl.uniprot.org/citations/17591906http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/17591906http://www.w3.org/2000/01/rdf-schema#comment"

Purpose

To identify and characterize gene expression changes associated with photoreceptor cell loss in a Bbs4-knockout mouse model of retinal degeneration.

Methods

Differential gene expression in the eyes of 5-month-old Bbs4(-/-) mice undergoing retinal degeneration were analyzed using gene microarrays (Affymetrix, Santa Clara, CA). Elevated ocular transcripts were confirmed by Northern blotting of RNA from Bbs4(-/-) and three additional mouse models of Bardet-Biedl Syndrome (BBS). TUNEL assays and transmission electron microscopy were used to study cell death and photoreceptor morphology in these mice.

Results

Three hundred fifty-four probes were differentially expressed in Bbs4(-/-) eyes compared with controls using a twofold cutoff. Numerous vision-related transcripts decreased because of photoreceptor cell loss. Increased expression of the stress response genes Edn2, Lcn2, Serpina3n, and Socs3 was noted at 5 months of age and as early as postnatal week 4 in the eyes of four BBS mouse model strains. A burst of apoptotic activity in the photoreceptor outer nuclear layer at postnatal week 2 and highly disorganized outer segments by postnatal weeks 4 to 6 was observed in all four strains.

Conclusions

The specific loss of photoreceptors in Bbs4(-)(/)(-) mice allows us to identify a set of genes that are preferentially expressed in photoreceptors compared with other cell types found in the eye and is a valuable resource in the continuing search for genes involved in retinal disease. The molecular and morphologic changes observed in young BBS animal model eyes implies that BBS proteins play a critical, early role in establishing the correct structure and function of photoreceptors."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.org/dc/terms/identifier"doi:10.1167/iovs.06-1477"xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/author"Huang J."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/author"Sheffield V.C."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/author"Stone E.M."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/author"Ross J.L."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/author"Mullins R.F."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/author"Nishimura D.Y."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/author"Swiderski R.E."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/author"Olvera M.A."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/date"2007"xsd:gYear
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/name"Invest Ophthalmol Vis Sci"xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/pages"3329-3340"xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/title"Gene expression analysis of photoreceptor cell loss in bbs4-knockout mice reveals an early stress gene response and photoreceptor cell damage."xsd:string
http://purl.uniprot.org/citations/17591906http://purl.uniprot.org/core/volume"48"xsd:string
http://purl.uniprot.org/citations/17591906http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/17591906
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