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Variabilities in retinal function and structure in a canine model of cone-rod dystrophy associated with RPGRIP1 support multigenic etiology.

Defects in cilia gene RPGRIP1 causes Leber congenital amaurosis and cone-rod dystrophy in humans. A form of the cone rod dystrophy dog ​​(cord1) was originally associated with the insertion of homozygotes in RPGRIP1 (RPGRIP1 in / in) as the major disease locus while homozygous deletion in MAP9 (MAP9 del / del) was identified as a modifier associated with early onset form. 

However, we find more variability in Electroretinograms cone (ERGs) ranging from normal to absent in the extended RPGRIP1 ins / ins colony of dogs, regardless Zebrafish Clia Kitsof genotype MAP9. Ophthalmoscopically, cone ERGabsent RPGRIP1 ins / ins eyes showing discoloration of the tapetal fundus with a variety of disease onset and development, while sd-OCT revealed atrophic changes. 

Despite a marked change in the cone ERG and retinal morphology, behavior photopic vision-guided comparable between the normal and the cones ERGabsent RPGRIP1 ins / ins littermates. Cone morphology less dog cone ERG cut with outdoor shortened and inner segments. Immunohistochemistry, cone ERGabsent RPGRIP1 ins / ins retina has a spacious L / M-opsin mislocalization, shortage CNGB3 label in L / M-cones, and less GC1 at all cones. Our results indicate that cord1 is a multigenic disease where mutations or MAP9 RPGRIP1 not only cause visual deficits, and additional gene (s) contributing to the functional and morphological defects specific cone.
Variabilities in retinal function and structure in a canine model of cone-rod dystrophy associated with RPGRIP1 support multigenic etiology.


OBJECTIVE
To determine whether banding secured with locking plastic clip splenic vein polymer on cadavers will lead to less CT imaging artifacts and achieve the equivalent mechanical strength, compared with plastic banding secured with metal clips blood vessels. ANIMALS 10 dead dogs. PROCEDURE clips of each ingredient are applied to each of the bodies in a Bovine Clia Kits crossover study. Triple-layer plastic band is secured with 4 medium-large or large polymer or metal clip is placed on the splenic vein cadavers and evaluated using CT. Beam-hardening artifacts artifacts rated by length, attenuation, and a subjective rating scale of 1 to 3 for mild to severe imaging artifacts. 

Guaranteed plastic band that is mechanically tested to determine the force-deformation curve and the yield strength. The findings for clip method compared with the 1-way ANOVA with Tukey post-test. RESULTS For metal clip, beam-hardening artifacts length and value of the subjective artifacts were significantly higher, while the value of attenuation significantly lowered, from the findings of polymer clips
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