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| Rd2 or retinal degeneration slow (rds) is a dominant spontaneous mutation in Prph2 (peripherin 2), mutant mice are characterized by slow degeneration of the outer nuclear layer of the retina beginning at 5 weeks, and eventual loss of retinal rod and cone cells by 12 months of age. This strain may be useful for studying human retinopathies. | |||||||||||||||
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- Disease & phenotype
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Description
Strain Information
Former Names C3A.BLiA-Pde6b+.O20-Prph2Rd2/J (Changed: 06-APR-12 ) C3A.BLiA-Pde6b+.O20-RdsRd2/J (Changed: 08-DEC-06 ) C3A.BLiA-Pde6b+.O20-Prph2Rd2 (Changed: 15-DEC-04 ) Type Congenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Mating System Homozygote x Homozygote (Female x Male) 01-MAR-06 Species laboratory mouse Donor Strain C57BL/LiA Generation ?+F69 (20-DEC-04)
Generation DefinitionsAppearance
agouti
Related Genotype: A/ADescription
Prph2 (peripherin 2) encodes a tetraspanning membrane protein found in the outer segment of both rod and cone photoreceptor cells, which is involved in photoreceptor disc morphogenesis. In this congenic strain, the retinal degeneration 2 (Prph2Rd2) allele was transferred from strain O20/A to C3A.BLiA-Pde6b+ (wild-type for retinal degeneration 1 - rd1). Rd2 or retinal degeneration slow (rds) is a dominant spontaneous mutation characterized by slow degeneration of the outer nuclear layer of the retina beginning at 5 weeks, loss of retinal rod cells by 7-10 months, loss of cone cells and all visual cell structures by 12 months of age, loss of some pigment epithelial cells and increased density of Muller cells accompanied by fibrillary tangles. This strain may be useful for studying human retinopathies.Development
This strain was developed in the laboratory of Dr. Willem J. De Grit at Erasmus Universiteit, Rotterdam, the Netherlands. The Prph2Rd2 allele was transferred from strain O20/A to C3A.BLiA-Pde6b+ via more than 20 backcross-intercross cycles (Schalken et al. 1990; S. Sanyal, personal communication). C3A.BLiA-Pde6b+ is a congenic strain having the wildtype Pde6b allele of C57BL/LiA on the C3Hf/HeA background. Since its arrival at The Jackson Laboratory, Stock No. 001979 has been maintained by sibling mating of mice homozygous for both Pde6b+ and Prph2Rd2.
Control Information
| Control | ||
|---|---|---|
| 001912 C3A.BLiA-Pde6b+/J | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying Pde6b+ allele
005252 B6EiC3Sn.BLiA-Ts(1716)65Dn/DnJ 003647 B6EiC3Sn.BLiAF1/J 002802 C3.BLiA Pde6b+-Krd/J 001912 C3A.BLiA-Pde6b+/J 003648 C3Sn.BLiA-Pde6b+/DnJ 004624 FVB.129P2-Pde6b+ Tyrc-ch Fmr1tm1Cgr/J 004828 FVB.129P2-Pde6b+ Tyrc-ch/AntJ 004808 STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J View Strains carrying Pde6b+ (8 strains)
001957 C3A Pde6brd1.O20/A-Prph2Rd2/J 001981 O20/A-Prph2Rd2/J
004821 C57BL/6J-Prph2Nmf193/J
Phenotype
Phenotype Information
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Retinitis Pigmentosa 7; RP7- Model with phenotypic similarity to human disease where etiologies are distinct. Human genes are associated with this disease. Orthologs of these genes do not appear in the mouse genotype(s).
Retinitis Pigmentosa; RP
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested. Choroidal Dystrophy, Central Areolar 2; CACD2 (PRPH2)
Fundus Albipunctatus (PRPH2)
Macular Dystrophy, Vitelliform, Adult-Onset (PRPH2)
Patterned Dystrophy of Retinal Pigment Epithelium (PRPH2)
assigned by genotype
Prph2Rd2/Prph2+
either: (involves: C3H * O20/A) or (involves: C57BL/LiA * O20/A)
- vision/eye phenotype
- abnormal photoreceptor outer segment morphology
- in pigmented heterozygous mice outer segments resemble those in albino mice but have more disc membranes that appear more tightly packed and less vacuolated (MGI Ref ID J:25582)
- nervous system phenotype
- abnormal photoreceptor outer segment morphology
- in pigmented heterozygous mice outer segments resemble those in albino mice but have more disc membranes that appear more tightly packed and less vacuolated (MGI Ref ID J:25582)
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Prph2Rd2/Prph2+
either: (involves: BALB/c * O20/A) or (involves: GR/A * O20/A) or (involves: O20/A * STS/A)
- vision/eye phenotype
- abnormal eye development
- at P14, considerable portions of the retinal surface have fewer outer segments and those present have disc structures that are irregularly oriented (MGI Ref ID J:25582)
- abnormal retinal layer morphology (MGI Ref ID J:25582)
- abnormal retinal photoreceptor morphology
- at 18 months, some photoreceptor synaptic terminals have multiple synaptic ribbons (MGI Ref ID J:25582)
- abnormal photoreceptor inner segment morphology
- occasional inner segments with swollen endoplasmic reticuli and vacuoles (MGI Ref ID J:25582)
- abnormal photoreceptor outer segment morphology
- outer segments are delayed in development but eventually appear throughout the retinal surface (MGI Ref ID J:25582)
- outer segments are reduced in length and morphologically abnormal appearing as round masses present from the apical end of the inner segments to the pigment epthelial villous processes (MGI Ref ID J:25582)
- these round masses contain irregular whorls of disc membranes that appear to be swollen and vacuolated (MGI Ref ID J:25582)
- at 18 months, outer segments become reduced and somewhat patchy (MGI Ref ID J:25582)
- increased retinal cone cell number
- the percentage of cones in the retina increases from 6 months on, with greater increases in the periphery than in the center of the retina (MGI Ref ID J:25582)
- abnormal retinal pigment epithelium morphology
- thin retinal outer nuclear layer
- maximal outer nuclear layer thickness is only 50-60% of wild-type (MGI Ref ID J:25582)
- at 18 months, outer nuclear layer thickness is markedly decreased (MGI Ref ID J:25582)
- thinning of the outer nuclear layer is first seen in the peripheral retina and later seen in the central retina (MGI Ref ID J:25582)
- nervous system phenotype
- abnormal retinal photoreceptor morphology
- at 18 months, some photoreceptor synaptic terminals have multiple synaptic ribbons (MGI Ref ID J:25582)
- abnormal photoreceptor inner segment morphology
- occasional inner segments with swollen endoplasmic reticuli and vacuoles (MGI Ref ID J:25582)
- abnormal photoreceptor outer segment morphology
- outer segments are delayed in development but eventually appear throughout the retinal surface (MGI Ref ID J:25582)
- outer segments are reduced in length and morphologically abnormal appearing as round masses present from the apical end of the inner segments to the pigment epthelial villous processes (MGI Ref ID J:25582)
- these round masses contain irregular whorls of disc membranes that appear to be swollen and vacuolated (MGI Ref ID J:25582)
- at 18 months, outer segments become reduced and somewhat patchy (MGI Ref ID J:25582)
- increased retinal cone cell number
- the percentage of cones in the retina increases from 6 months on, with greater increases in the periphery than in the center of the retina (MGI Ref ID J:25582)
- pigmentation phenotype
- abnormal retinal pigment epithelium morphology
Prph2Rd2/Prph2+
either: C.O20-Prph2Rd2 or C3.O20-Prph2Rd2
- vision/eye phenotype
- abnormal Muller cell morphology
- thin retinal outer nuclear layer
- at 16 months, the outer nuclear layer is reduced to 3 to 4 rows (MGI Ref ID J:27850)
- nervous system phenotype
- abnormal Muller cell morphology
Prph2Rd2/Prph2Rd2
O20/A-Prph2Rd2
- vision/eye phenotype
- abnormal eye electrophysiology
- smaller a-wave and b-wave amplitudes at all intensities tested at 1 and 2-3 months of age compared to age matched controls (MGI Ref ID J:7600)
- time to peak of the waves is increased at 2-3 months of age but not at 1 month of age compared to age matched controls (MGI Ref ID J:7600)
- absence of detectable a-wave at all intensities tested at 6-7 and 13 months of age (MGI Ref ID J:7600)
- b-wave amplitudes are reduced but still detectable at higher intensities at 6-7 months of age (MGI Ref ID J:7600)
- abnormal retina morphology
- thin with distorted structure at 12 months of age (MGI Ref ID J:6432)
- abnormal retinal development
- abnormal retinal neuronal layer morphology
- at 9 months of age in some areas the outermost row of the inner nuclear layer has merged with the outer nuclear layer (MGI Ref ID J:6432)
- abnormal retinal outer nuclear layer morphology
- maximal thickness is detected on P7 compared to P11 in wild-type controls (MGI Ref ID J:6432)
- at 9 months of age a single row of outer nuclei is present in the centrally located region surrounding the optic nerve head and in the most outlying region approaching the periphery, while in areas lying between these two regions, two or three rows of nuclei are seen in the outer layer (MGI Ref ID J:6432)
- retinal outer nuclear layer degeneration
- thin retinal outer nuclear layer (MGI Ref ID J:7600)
- abnormal retinal photoreceptor layer morphology
- abnormal retinal photoreceptor morphology
- abnormal retinal cone cell morphology
- absent retinal cone cells
- at 12 months of age (MGI Ref ID J:6432)
- abnormal retinal rod cell outer segment morphology
- the layer of rod outer segments fails to develop (MGI Ref ID J:6432)
- absent photoreceptor outer segment
- retinal photoreceptor cells lack outer segments (MGI Ref ID J:75095)
- absent retinal rod cells
- thin retinal outer plexiform layer
- abnormal retinal pigment epithelium morphology
- retinal degeneration
- slight degenerative changes are seen in the outer nuclear layer starting at 5 weeks (MGI Ref ID J:6051)
- degeneration slowly progresses until at 7-10 months rods are absent and the photoreceptor cell nuclei are reduced to 1 row (MGI Ref ID J:6051)
- detectable at P14 as a decrease in the thickness of the outer nuclear layer (MGI Ref ID J:6432)
- by 28 days of age thinning of the photoreceptor and outer plexiform layers is seen (MGI Ref ID J:6432)
- at 9 months of age extensive areas of the retina completely lack visual cells (MGI Ref ID J:6432)
- at 12 months of age, all visual cell structures are gone and degenerative changes are observed in other layers of the retina (MGI Ref ID J:6432)
- at 18-24 months of age continuing degeneration results in further distortion and thinning of the retina (MGI Ref ID J:6432)
- retinal neovascularization
- extensive vascularization at 12 months of age (MGI Ref ID J:6432)
- immune system phenotype
- abnormal macrophage morphology
- at P11 macrophages in the retina remain large and display high levels of glucosaminidase activity (MGI Ref ID J:6432)
- as the outer nuclear layer thins, fewer macrophages are detected in this layer of the retina (MGI Ref ID J:6432)
- at 12 months of age only a few small macrophages remain in the outer aspect of the inner nuclear layer (MGI Ref ID J:6432)
- nervous system phenotype
- abnormal retinal photoreceptor morphology
- abnormal retinal cone cell morphology
- absent retinal cone cells
- at 12 months of age (MGI Ref ID J:6432)
- abnormal retinal rod cell outer segment morphology
- the layer of rod outer segments fails to develop (MGI Ref ID J:6432)
- absent photoreceptor outer segment
- retinal photoreceptor cells lack outer segments (MGI Ref ID J:75095)
- absent retinal rod cells
- pigmentation phenotype
- abnormal retinal pigment epithelium morphology
- cardiovascular system phenotype
- retinal neovascularization
- extensive vascularization at 12 months of age (MGI Ref ID J:6432)
- hematopoietic system phenotype
- abnormal macrophage morphology
- at P11 macrophages in the retina remain large and display high levels of glucosaminidase activity (MGI Ref ID J:6432)
- as the outer nuclear layer thins, fewer macrophages are detected in this layer of the retina (MGI Ref ID J:6432)
- at 12 months of age only a few small macrophages remain in the outer aspect of the inner nuclear layer (MGI Ref ID J:6432)
Prph2Rd2/Prph2Rd2
either: C.O20-Prph2Rd2 or C3.O20-Prph2Rd2
- vision/eye phenotype
- abnormal Muller cell morphology
- at P11 small numbers of Muller cells and radial fibers are found in the outer plexiform layer (MGI Ref ID J:27850)
- at P28 the number of Muller cells is increased, the cells tend to be unevenly distributed, and occasional fibrillar tangles are seen in the inner plexiform layer (MGI Ref ID J:27850)
- at 2 months of age the density of Muller cells has increased dramatically, in the periphery these cells have fibers that penetrate the outer nuclear layer and cover it externally, and the number of fibrillar tangles is increased (MGI Ref ID J:27850)
- at 9 months of age the density of Muller cells, fibrillar tangles, and horizontal fibers in the plexiform layers is further increased (MGI Ref ID J:27850)
- thin retinal outer nuclear layer
- nervous system phenotype
- abnormal Muller cell morphology
- at P11 small numbers of Muller cells and radial fibers are found in the outer plexiform layer (MGI Ref ID J:27850)
- at P28 the number of Muller cells is increased, the cells tend to be unevenly distributed, and occasional fibrillar tangles are seen in the inner plexiform layer (MGI Ref ID J:27850)
- at 2 months of age the density of Muller cells has increased dramatically, in the periphery these cells have fibers that penetrate the outer nuclear layer and cover it externally, and the number of fibrillar tangles is increased (MGI Ref ID J:27850)
- at 9 months of age the density of Muller cells, fibrillar tangles, and horizontal fibers in the plexiform layers is further increased (MGI Ref ID J:27850)
Prph2Rd2/Prph2Rd2
involves: BALB/c * O20/AThis mouse can be used to support research in many areas including:Pde6b+ related
Prph2Rd2 relatedSensorineural Research
Retinal Degeneration
wild-type
Research Tools
Sensorineural Research
retinal degeneration
Sensorineural Research
Retinal Degeneration
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Pde6b+ | ||
|---|---|---|---|
| Allele Name | wild type | ||
| Allele Type | Not Applicable | ||
| Mutation Made By | Frank Kooy, University of Antwerp | ||
| Gene Symbol and Name | Pde6b, phosphodiesterase 6B, cGMP, rod receptor, beta polypeptide | ||
| Chromosome | 5 | ||
| Gene Common Name(s) | CSNB3; CSNBAD2; PDEB; Pdeb; RP40; nmf137; phosphodiesterase, cGMP, rod receptor, beta polypeptide; r; rd; rd-1; rd1; rd10; retinal degeneration; retinal degeneration 1; retinal degeneration 10; | ||
| Allele Symbol | Prph2Rd2 | ||
| Allele Name | retinal degeneration 2 | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | Prph2Rds; Rd-2; Rds; RdsRd2; rds-; retinal degeneration slow; | ||
| Strain of Origin | O20/A | ||
| Gene Symbol and Name | Prph2, peripherin 2 | ||
| Chromosome | 17 | ||
| Gene Common Name(s) | AOFMD; AVMD; CACD2; DS; Nmf193; PRPH; RDS; RP7; RSRDS; Rd-2; Rd2; Rds; TSPAN22; neuroscience mutagenesis facility, 193; retinal degeneration 2; retinal degeneration, slow; retinal degeneration, slow (retinitis pigmentosa 7); | ||
| Molecular Note | The mutation is an insertion of approximately 10 kb in the gene after nucleotide 899 (numbering of the encoded mRNA), disrupting the protein coding sequence in exon 2. The inserted DNA was similar to both the TSE of mice, repeated elements found in the H2 complex, and to the mouse early transposon (ETn). Northern blot analysis demonstrated that an aberrant 12 kb transcript was produced from this allele, although at reduced levels compared to wild-type. This allele is predicted to encode a truncated protein with its carboxy terminal 116 amino acids replaced by 35 amino acids from sequences in the insertion. Mutant mice doubly homozygous for two retinal degeneration mutations (Pde6brd1 and RdsRd2) shows an intermediate level of mRNAs for the beta subunit of cGMP-PDE and for several other phototransduction related proteins, suggesting an interaction between Pde6brd1 and RdsRd2. [MGI Ref ID J:2579] [MGI Ref ID J:9635] | ||
References
References provided by MGI
Selected Reference(s)
Schalken JJ; Janssen JJ; Sanyal S; Hawkins RK; de Grip WJ. 1990. Development and degeneration of retina in rds mutant mice: immunoassay of the rod visual pigment rhodopsin. Biochim Biophys Acta 1033(1):103-9. [PubMed: 2137350] [MGI Ref ID J:109932]
Additional References
Chang B; Hawes NL; Hurd RE; Davisson MT; Nusinowitz S; Heckenlively JR. 2002. Retinal degeneration mutants in the mouse. Vision Res 42(4):517-25. [PubMed: 11853768] [MGI Ref ID J:75095]
Pde6b+ relatedPrph2Rd2 relatedDobkin C; Rabe A; Dumas R; El Idrissi A; Haubenstock H; Brown WT. 2000. Fmr1 knockout mouse has a distinctive strain-specific learning impairment. Neuroscience 100(2):423-9. [PubMed: 11008180] [MGI Ref ID J:119166]
Ivanco TL; Greenough WT. 2002. Altered mossy fiber distributions in adult Fmr1 (FVB) knockout mice. Hippocampus 12(1):47-54. [PubMed: 11918288] [MGI Ref ID J:113177]
Sakamoto K; McCluskey M; Wensel TG; Naggert JK; Nishina PM. 2009. New mouse models for recessive retinitis pigmentosa caused by mutations in the Pde6a gene. Hum Mol Genet 18(1):178-92. [PubMed: 18849587] [MGI Ref ID J:142108]
Zhao MG; Toyoda H; Ko SW; Ding HK; Wu LJ; Zhuo M. 2005. Deficits in trace fear memory and long-term potentiation in a mouse model for fragile X syndrome. J Neurosci 25(32):7385-92. [PubMed: 16093389] [MGI Ref ID J:100197]
Agarwal N. 1994. Diurnal expression of NGF1-A mRNA in retinal degeneration slow (rds) mutant mouse retina. FEBS Lett 339(3):253-7. [PubMed: 8112464] [MGI Ref ID J:16898]
Agarwal N; Jomary C; Jones SE; O'Rourke K; Chaitin M; Wordinger RJ; Murphy BF. 1996. Immunocytochemical colocalization of clusterin in apoptotic photoreceptor cells in retinal degeneration slow rds mutant mouse retinas. Biochem Biophys Res Commun 225(1):84-91. [PubMed: 8769098] [MGI Ref ID J:34976]
Agarwal N; Nir I; Papermaster DS. 1994. Loss of diurnal arrestin gene expression in rds mutant mouse retinas. Exp Eye Res 58(1):1-8. [PubMed: 8157095] [MGI Ref ID J:28768]
Ali RR; Reichel MB; Kanuga N; Munro PM; Alexander RA; Clarke AR; Luthert PJ; Bhattacharya SS; Hunt DM. 1998. Absence of p53 delays apoptotic photoreceptor cell death in the rds mouse. Curr Eye Res 17(9):917-23. [PubMed: 9746439] [MGI Ref ID J:111968]
Ali RR; Sarra GM; Stephens C; Alwis Md; Bainbridge JW; Munro PM; Fauser S; Reichel MB; Kinnon C; Hunt DM; Bhattacharya SS; Thrasher AJ. 2000. Restoration of photoreceptor ultrastructure and function in retinal degeneration slow mice by gene therapy Nat Genet 25(3):306-10. [PubMed: 10888879] [MGI Ref ID J:63124]
Anderson RE; Maude MB; Bok D. 2001. Low docosahexaenoic acid levels in rod outer segment membranes of mice with rds/peripherin and P216L peripherin mutations. Invest Ophthalmol Vis Sci 42(8):1715-20. [PubMed: 11431433] [MGI Ref ID J:70247]
Barber AC; Hippert C; Duran Y; West EL; Bainbridge JW; Warre-Cornish K; Luhmann UF; Lakowski J; Sowden JC; Ali RR; Pearson RA. 2013. Repair of the degenerate retina by photoreceptor transplantation. Proc Natl Acad Sci U S A 110(1):354-9. [PubMed: 23248312] [MGI Ref ID J:192521]
Cayouette M; Behn D; Sendtner M; Lachapelle P; Gravel C. 1998. Intraocular gene transfer of ciliary neurotrophic factor prevents death and increases responsiveness of rod photoreceptors in the retinal degeneration slow mouse. J Neurosci 18(22):9282-93. [PubMed: 9801367] [MGI Ref ID J:50901]
Cayouette M; Smith SB; Becerra SP; Gravel C. 1999. Pigment epithelium-derived factor delays the death of photoreceptors in mouse models of inherited retinal degenerations. Neurobiol Dis 6(6):523-32. [PubMed: 10600408] [MGI Ref ID J:59343]
Chaitin MH; Ankrum MT; Wortham HS. 1996. Distribution of CD44 in the retina during development and the rds degeneration. Brain Res Dev Brain Res 94(1):92-8. [PubMed: 8816281] [MGI Ref ID J:33822]
Chakraborty D; Conley SM; Stuck MW; Naash MI. 2010. Differences in RDS trafficking, assembly and function in cones versus rods: insights from studies of C150S-RDS. Hum Mol Genet 19(24):4799-812. [PubMed: 20858597] [MGI Ref ID J:166363]
Chakraborty D; Ding XQ; Conley SM; Fliesler SJ; Naash MI. 2009. Differential requirements for retinal degeneration slow intermolecular disulfide-linked oligomerization in rods versus cones. Hum Mol Genet 18(5):797-808. [PubMed: 19050038] [MGI Ref ID J:145008]
Chang B; Hawes NL; Hurd RE; Davisson MT; Nusinowitz S; Heckenlively JR. 2002. Retinal degeneration mutants in the mouse. Vision Res 42(4):517-25. [PubMed: 11853768] [MGI Ref ID J:75095]
Chang B; Hawes NL; Hurd RE; Wang J; Howell D; Davisson MT; Roderick TH; Nusinowitz S; Heckenlively JR. 2005. Mouse models of ocular diseases. Vis Neurosci 22(5):587-93. [PubMed: 16332269] [MGI Ref ID J:156373]
Chang B; Heckenlively JR; Hawes NL; Roderick TH. 1993. New mouse primary retinal degeneration (rd-3). Genomics 16(1):45-9. [PubMed: 8486383] [MGI Ref ID J:4367]
Cheng T; Peachey NS; Li S; Goto Y; Cao Y; Naash MI. 1997. The effect of peripherin/rds haploinsufficiency on rod and cone photoreceptors. J Neurosci 17(21):8118-28. [PubMed: 9334387] [MGI Ref ID J:43692]
Conley S; Nour M; Fliesler SJ; Naash MI. 2007. Late-onset cone photoreceptor degeneration induced by R172W mutation in Rds and partial rescue by gene supplementation. Invest Ophthalmol Vis Sci 48(12):5397-407. [PubMed: 18055786] [MGI Ref ID J:132515]
Connell G; Bascom R; Molday L; Reid D; McInnes RR; Molday RS. 1991. Photoreceptor peripherin is the normal product of the gene responsible for retinal degeneration in the rds mouse. Proc Natl Acad Sci U S A 88(3):723-6. [PubMed: 1992463] [MGI Ref ID J:10978]
Demos C; Bandyopadhyay M; Rohrer B. 2008. Identification of candidate genes for human retinal degeneration loci using differentially expressed genes from mouse photoreceptor dystrophy models. Mol Vis 14:1639-49. [PubMed: 18776951] [MGI Ref ID J:140115]
Ding XQ; Nour M; Ritter LM; Goldberg AF; Fliesler SJ; Naash MI. 2004. The R172W mutation in peripherin/rds causes a cone-rod dystrophy in transgenic mice. Hum Mol Genet 13(18):2075-87. [PubMed: 15254014] [MGI Ref ID J:92815]
Ekstrom P; Sanyal S; Narfstrom K; Chader GJ; van Veen T. 1988. Accumulation of glial fibrillary acidic protein in Muller radial glia during retinal degeneration. Invest Ophthalmol Vis Sci 29(9):1363-71. [PubMed: 3417421] [MGI Ref ID J:27850]
Farjo R; Fliesler SJ; Naash MI. 2007. Effect of Rds abundance on cone outer segment morphogenesis, photoreceptor gene expression, and outer limiting membrane integrity. J Comp Neurol 504(6):619-30. [PubMed: 17722028] [MGI Ref ID J:131880]
Farjo R; Skaggs JS; Nagel BA; Quiambao AB; Nash ZA; Fliesler SJ; Naash MI. 2006. Retention of function without normal disc morphogenesis occurs in cone but not rod photoreceptors. J Cell Biol 173(1):59-68. [PubMed: 16585269] [MGI Ref ID J:107832]
Fletcher RT; Sanyal S; Krishna G; Aguirre G; Chader GJ. 1986. Genetic expression of cyclic GMP phosphodiesterase activity defines abnormal photoreceptor differentiation in neurological mutants of inherited retinal degeneration. J Neurochem 46(4):1240-5. [PubMed: 3005510] [MGI Ref ID J:12044]
Franke H; Klimke K; Brinckmann U; Grosche J; Francke M; Sperlagh B; Reichenbach A; Liebert UG; Illes P. 2005. P2X(7) receptor-mRNA and -protein in the mouse retina; changes during retinal degeneration in BALBCrds mice. Neurochem Int 47(4):235-42. [PubMed: 15964665] [MGI Ref ID J:103892]
Gao H; Hollyfield JG. 1996. Basic fibroblast growth factor: increased gene expression in inherited and light-induced photoreceptor degeneration. Exp Eye Res 62(2):181-9. [PubMed: 8698078] [MGI Ref ID J:32161]
Hawes NL; Smith RS; Chang B; Davisson M; Heckenlively JR; John SW. 1999. Mouse fundus photography and angiography: a catalogue of normal and mutant phenotypes. Mol Vis 5:22. [PubMed: 10493779] [MGI Ref ID J:59481]
Hawkins RK; Jansen HG; Sanyal S. 1985. Development and degeneration of retina in rds mutant mice: photoreceptor abnormalities in the heterozygotes. Exp Eye Res 41(6):701-20. [PubMed: 3830736] [MGI Ref ID J:25582]
Heckenlively JR; Chang B; Erway LC; Peng C; Hawes NL; Hageman GS; Roderick TH. 1995. Mouse model for Usher syndrome: linkage mapping suggests homology to Usher type I reported at human chromosome 11p15. Proc Natl Acad Sci U S A 92(24):11100-4. [PubMed: 7479945] [MGI Ref ID J:121993]
Hilgers J. 1979. Prph2<Rd2> - retinal degeneration-2 Mouse News Lett 60:38. [MGI Ref ID J:65320]
Horio N; Kachi S; Hori K; Okamoto Y; Yamamoto E; Terasaki H; Miyake Y. 2001. Progressive change of optical coherence tomography scans in retinal degeneration slow mice. Arch Ophthalmol 119(9):1329-32. [PubMed: 11545639] [MGI Ref ID J:115731]
Hughes EH; Schlichtenbrede FC; Murphy CC; Sarra GM; Luthert PJ; Ali RR; Dick AD. 2003. Generation of activated sialoadhesin-positive microglia during retinal degeneration. Invest Ophthalmol Vis Sci 44(5):2229-34. [PubMed: 12714665] [MGI Ref ID J:83027]
Iandiev I; Biedermann B; Bringmann A; Reichel MB; Reichenbach A; Pannicke T. 2006. Atypical gliosis in Muller cells of the slowly degenerating rds mutant mouse retina. Exp Eye Res 82(3):449-57. [PubMed: 16154566] [MGI Ref ID J:106873]
Jansen HG; Sanyal S. 1984. Development and degeneration of retina in rds mutant mice: electron microscopy. J Comp Neurol 224(1):71-84. [PubMed: 6715580] [MGI Ref ID J:7415]
Jones BW; Watt CB; Frederick JM; Baehr W; Chen CK; Levine EM; Milam AH; Lavail MM; Marc RE. 2003. Retinal remodeling triggered by photoreceptor degenerations. J Comp Neurol 464(1):1-16. [PubMed: 12866125] [MGI Ref ID J:84675]
Kedzierski W; Lloyd M; Birch DG; Bok D; Travis GH. 1997. Generation and analysis of transgenic mice expressing P216L-substituted rds/peripherin in rod photoreceptors. Invest Ophthalmol Vis Sci 38(2):498-509. [PubMed: 9040483] [MGI Ref ID J:112440]
Kedzierski W; Nusinowitz S; Birch D; Clarke G; McInnes RR; Bok D; Travis GH. 2001. Deficiency of rds/peripherin causes photoreceptor death in mouse models of digenic and dominant retinitis pigmentosa. Proc Natl Acad Sci U S A 98(14):7718-23. [PubMed: 11427722] [MGI Ref ID J:77309]
Krishnamoorthy R; Agarwal N; Chaitin MH. 2000. Upregulation of CD44 expression in the retina during the rds degeneration. Brain Res Mol Brain Res 77(1):125-30. [PubMed: 10814838] [MGI Ref ID J:62192]
LaVail MW; Yasumura D; Matthes MT; Lau-Villacorta C; Unoki K; Sung CH; Steinberg RH. 1998. Protection of mouse photoreceptors by survival factors in retinal degenerations. Invest Ophthalmol Vis Sci 39(3):592-602. [PubMed: 9501871] [MGI Ref ID J:46230]
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Health & husbandry
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Mating System Homozygote x Homozygote (Female x Male) 01-MAR-06
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Live Mice
Price per mouse (US dollars $) Gender Genotypes Provided Individual Mouse $121.75 Female or Male Homozygous for Prph2Rd2
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Live Mice
Price per mouse (US dollars $) Gender Genotypes Provided Individual Mouse $158.30 Female or Male Homozygous for Prph2Rd2
Price per Pair (US dollars $) Pair Genotype $316.60 Homozygous for Prph2Rd2 x Homozygous for Prph2Rd2 Standard Supply
Research Strain. Availability determined by The Jackson Laboratory scientist holding the strain.
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Standard Supply
Research Strain. Availability determined by The Jackson Laboratory scientist holding the strain.
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