RGD Reference Report - Development of a Pde6b Gene Knockout Rat Model for Studies of Degenerative Retinal Diseases.

	Submit Data \| Help \| Video Tutorials \| News \| Publications \| Download \| REST API \| Citing RGD \| Contact
	Search RGD Grant Resources Citing RGD About Us Contact Us Genes QTLs Strains Markers Genome Information Ontologies Cell Lines References Download Submit Data OntoMate (Literature Search) JBrowse (Genome Browser) Synteny Browser (VCMap) (beta) Variant Visualizer Multi-Ontology Enrichment (MOET) Gene-Ortholog Location Finder (GOLF) InterViewer (Protein-Protein Interactions) PhenoMiner (Quatitative Phenotypes) Gene Annotator OLGA (Gene List Generator) AllianceMine GViewer (Genome Viewer) Overgo Probe Designer Aging & Age-Related Disease Cancer & Neoplastic Disease Cardiovascular Disease COVID-19 Developmental Disease Diabetes Hematologic Disease Immune & Inflammatory Disease Infectious Disease Liver Disease Neurological Disease Obesity & Metabolic Syndrome Renal Disease Respiratory Disease Sensory Organ Disease Find Models new Genetic Models Autism Models Rat PhenoMiner (Quantitative Phenotypes) Chinchilla PhenoMiner Expected Ranges (Quantitative Phenotype) PhenoMiner Term Comparison Hybrid Rat Diversity Panel Phenotypes GERRC (Gene Editing Rat Resource Center) Phenotypes in Other Animal Models Animal Husbandry Strain Medical Records Phylogenetics Strain Availability Calendar Rats 101 Submissions Photo Archive Pathways Rat Community Forum Directory of Rat Laboratories Video Tutorials News RGD Publications RGD Poster Archive Nomenclature Guidelines Resource Links Laboratory Resources Employment Resources
	Advanced Search (OLGA)

General

Development of a Pde6b Gene Knockout Rat Model for Studies of Degenerative Retinal Diseases.
Authors:	Yeo, Joon Hyung Jung, Bok Kyoung Lee, Heuiran Baek, In-Jeoung Sung, Young Hoon Shin, Hae-Sol Kim, Hong Kyung Seo, Kyoung Yul Lee, Joo Yong
Citation:	Yeo JH, etal., Invest Ophthalmol Vis Sci. 2019 Apr 1;60(5):1519-1526. doi: 10.1167/iovs.18-25556.
RGD ID:	40924664
Pubmed:	PMID:31009522 (View Abstract at PubMed)
DOI:	DOI:10.1167/iovs.18-25556 (Journal Full-text)
Purpose: To describe the phenotypes of a newly developed Pde6b-deficient rat model of retinal degeneration. Methods: Pde6b knockout rats were produced by CRISPR-Cpf1 technology. Pde6b knockout rats were evaluated for ocular abnormalities by comparison with wild-type eyes. Eyes were imaged using fundus photography and optical coherence tomography (OCT), stained by hematoxylin and eosin (H&E), and examined by TUNEL assay. Finally, eyes were functionally assessed by electroretinograms (ERGs). Results: Pde6b knockout rats exhibited visible photoreceptor degeneration at 3 weeks of postnatal age. The fundus appearance of mutants was notable for pigmentary changes, vascular attenuation with an irregular vascular pattern, and outer retinal thinning, which resembled retinitis pigmentosa (RP) in humans. OCT showed profound retinal thinning in Pde6b knockout rats; the outer nuclear layer (ONL) was significantly thinner in Pde6b knockout rats, with relative preservation of the inner retina at 3 weeks of postnatal age. H&E staining confirmed extensive degeneration of the ONL, beginning at 3 weeks of postnatal age; no ONL remained in the retina by 16 weeks of postnatal age. Retinal sections of Pde6b knockout rats were highly positive for TUNEL, specifically in the ONL. In ERGs, Pde6b knockout rats showed no detectable a- or b-waves at 8 weeks of postnatal age. Conclusions: The Pde6b knockout rat exhibits photoreceptor degeneration. It may provide a better model for experimental therapy for RP because of its slower progression and larger anatomic architecture than the corresponding mouse model. Further studies in this rat model may yield insights into effective therapies for human RP.

Annotation Click to see Annotation Detail View

Disease Annotations

retinitis pigmentosa (IMP,ISO)

Gene Ontology Annotations

Biological Process

retinal cell apoptotic process (IMP)

Phenotype Annotations

Mammalian Phenotype

abnormal retina blood vessel pattern (IMP)

increased retina apoptosis (IMP)

retina degeneration (IMP)

retina outer nuclear layer degeneration (IMP)

retina photoreceptor degeneration (IMP)

thin retina outer nuclear layer (IMP)

Objects Annotated

Genes (Rattus norvegicus)

Pde6b (phosphodiesterase 6B)

Pde6b^em1Baek (phosphodiesterase 6B; Cpf1-CRISPR induced mutant1, Baek)

Genes (Mus musculus)

Pde6b (phosphodiesterase 6B, cGMP, rod receptor, beta polypeptide)

Genes (Homo sapiens)

PDE6B (phosphodiesterase 6B)

Strains

SD-Pde6b^em1Baek (NA)

Additional Information

Gene Annotator (Functional Annotation) unavailable	Gene Annotator (Annotation Distribution) unavailable	Variant Visualizer (Genomic Variants) unavailble Variant Visualizer (Genomic Variants) unavailable
Gene Annotator (Functional Annotation)	Gene Annotator (Annotation Distribution)	Variant Visualizer (Genomic Variants)

InterViewer (Protein-Protein Interactions) unavailable	Gviewer (Genome Viewer) unavailable	Variant Visualizer (Damaging Variants) unavailble Variant Visualizer (Damaging Variants) unavailable
InterViewer (Protein-Protein Interactions)	GViewer (Genome Viewer)	Variant Visualizer (Damaging Variants)

Gene Annotator (Annotation Comparison) unavailable	OLGA (Gene List Generator) unavailable
Gene Annotator (Annotation Comparison)	OLGA (Gene List Generator)	Excel (Download)

MOET (Multi-Ontology Enrichement) unavailable	GOLF (Gene-Ortholog Location Finder) unavailable
MOET (Multi-Ontology Enrichement)	GOLF (Gene-Ortholog Location Finder)

Create Name:

Description:

Send us a Message

Development of a Pde6b Gene Knockout Rat Model for Studies of Degenerative Retinal Diseases.

Mammalian Phenotype