Retina International's Scientific Newsletter - Animal Model Database

	Retina International's Scientific Newsletter
	Animal Model Database Rattus norvegicus

Recent update from: 13.11.2002

You are always welcome to give your comments.

Species: Rattus norvegicus Race: Occurrence of the model: transgenic	Disease: ADRP Inheritance: ad Assignment: Gene: Rho Mutation(s): S334X
Phenotype, morphological
Phenotype, clinic
Phenotype, electrophysiological
Phenotype, biochemical
Remarks Rho in inappropriately high levels in the plasma membrane and cytoplasm Misrouting of Rho Effect at mutation ratios of at least 10% At P15 3.9% of photoreceptors/d are lost at P90 only 2.4%/d are lost
Human counterpart: ADRP Human disease:	Reference: (2) Online Reference: Online Data:

Species: Rattus norvegicus Race: RCS Occurrence of the model: naturally	Disease: rdy Inheritance: ar Assignment: RN3q35-36, D3Rat256 Gene: c-mer Mutation(s): Deletion 419 bp in 5' part
Phenotype, morphological
Phenotype, clinic
Phenotype, electrophysiological
Phenotype, biochemical
Remarks
Human counterpart: MERTK Human disease: ARRP	Reference: (4) Online Reference: Online Data:

Species: Rattus norvegicus Race: RCS Occurrence of the model: naturally	Disease: rdy^- Inheritance: Assignment: D3Mit12-[0.99]-D3Mit13/14-D3Rat161/257-rdy-D3Rat258-D3Rat101-[0.89]-D3Rat160-[1.6]-D3Rat126 Gene: mertk Mutation(s): Deletion of exon 2 including the splice acceptor
Phenotype, morphological Failure of RPE to phagocytose the OS shed completely in a 9 d rythm Progressive postnatal loss of photoreceptors Accumulation of OS debris between PCL and RPE Phagocytose defect is particle specific since latex beads are ingested
Phenotype, clinic Shed POS material accumulates in subretinal space Onsetat P20 and nearly complete loss of retinal nuclei at P16
Phenotype, electrophysiological
Phenotype, biochemical Defect of RPE to phagocytose ROS
Remarks Reduced levels of aA-crystallins Reduced level of rhok Injection of AV carrying mertk preserves photoreceptors surrounding the injection site
Human counterpart: MERTK Human disease: ARRP	Reference: (3) (1) (5) Online Reference: Online Data:

References

D'Cruz,P.M., Yasumura,D., Weir,J., Matthes,M.T., Abderrahim,H., LaVail,M.M., and Vollrath,D. Mutation of the receptor tyrosine kinase gene Mertk in the retinal dystrophic RCS rat. 2000; Hum.Mol.Genet. 9: 645-651.
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Green,E.S., Menz,M.D., LaVail,M.M., and Flannery,J.G. Characterization of rhodopsin mis-sorting and constitutive activation in a transgenic rat model of retinitis pigmentosa. 2000; Invest.Ophthalmol.Vis.Sci. 41: 1546-1553.
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Maeda,A., Ohguro,H., Maeda,T., Nakagawa,T., and Kuroki,Y. Low expression of alphaA-crystallins and rhodopsin kinase of photoreceptors in retinal dystrophy rat. 1999; Invest.Ophthalmol.Vis.Sci. 40: 2788-2794.
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Nandrot,E., Dufour,E.M., Provost,A.C., Pequignot,M.O., Bonnel,S., Gogat,K., Marchant,D., Rouillac,C., Sepulchre,d.C., Bihoreau,M.T., Shaver,C., Dufier,J.L., Marsac,C., Lathrop,M., Menasche,M., and Abitbol,M.M. Homozygous deletion in the coding sequence of the c-mer gene in RCS rats unravels general mechanisms of physiological cell adhesion and apoptosis. 2000; Neurobiol.Dis. 7: 586-599.
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Vollrath,D., Feng,W., Duncan,J.L., Yasumura,D., D'Cruz,P.M., Chappelow,A., Matthes,M.T., Kay,M.A., and LaVail,M.M. Correction of the retinal dystrophy phenotype of the RCS rat by viral gene transfer of Mertk. 2001; Proc.Natl.Acad.Sci.U.S.A. 98: 12584-12589.
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Contact the editor		This site is maintained and edited by Dr. rer. medic. Markus Preising, Dipl.Biol. Molecular Genetics Laboratory Department of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics University of Regensburg Head: Prof. Dr. med. Birgit Lorenz