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Phenotypes Associated with This Genotype
Genotype
MGI:4360797
Allelic
Composition
Tg(RBP3-ELOVL4*)2Kzh/0
Genetic
Background
C57BL/6-Tg(RBP3-ELOVL4*)2Kzh
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
vision/eye
• abnormalities are more evident as expression levels of the transgene increase in the three lines created
• mice exhibit localized regions of the retina that appear lighter because of depigmentation in areas of retinal pigment epithelium atrophy and photoreceptor degeneration
• in the central retina near the optic nerve head at 7 months
• in the near-peripheral retina at 7 months
• in the near-peripheral retina at 7 months
• in the central and near peripheral retina near the optic nerve head at 7 months
• in the far peripheral retina at 7 months, outer segment length is 50% less than in wild-type mice
• in the midperipheral retina at 7 months, outer segment lengths are reduced and debris is found in the subretinal space unlike in wild-type mice
• at 16 weeks, 50% of photoreceptor in the outer nuclear layer are degenerated
• in the far peripheral retina at 7 months, the outer nuclear layer contains only 5 to 6 rows of photoreceptor nuclei compared with 10 to 12 in wild-type mice
• in the midperipheral retina at 7 months, the outer nuclear layer is disorganized and reduced to 3 to 4 rows unlike in wild-type mice
• more severe in the center of the retina
• in the far peripheral retina at 7 months, the outer nuclear layer contains only 5 to 6 rows of photoreceptor nuclei compared with 10 to 12 in wild-type mice
• in the midperipheral retina at 7 months, the outer nuclear layer is disorganized and reduced to 3 to 4 rows unlike in wild-type mice
• mice exhibit a progressive loss of photoreceptor nuclei from the periphery to the center unlike in wild-type mice
• obliterated in the central retina near the optic nerve head at 7 months
• progressive, localized, and more severe in the center of the retina
• at rod-saturation, cone b-wave declines in amplitude compared to in wild-type mice
• at 84 weeks, rod b-wave response is absent

nervous system
• in the central retina near the optic nerve head at 7 months
• in the near-peripheral retina at 7 months
• in the near-peripheral retina at 7 months
• in the central and near peripheral retina near the optic nerve head at 7 months
• in the far peripheral retina at 7 months, outer segment length is 50% less than in wild-type mice
• in the midperipheral retina at 7 months, outer segment lengths are reduced and debris is found in the subretinal space unlike in wild-type mice
• at 16 weeks, 50% of photoreceptor in the outer nuclear layer are degenerated
• in the far peripheral retina at 7 months, the outer nuclear layer contains only 5 to 6 rows of photoreceptor nuclei compared with 10 to 12 in wild-type mice
• in the midperipheral retina at 7 months, the outer nuclear layer is disorganized and reduced to 3 to 4 rows unlike in wild-type mice

pigmentation
• more severe in the center of the retina
• in the retinal pigmented epithelium at 7 months

Mouse Models of Human Disease
OMIM ID Ref(s)
Stargardt Disease 3; STGD3 600110 J:97173


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
05/24/2016
MGI 6.04
The Jackson Laboratory