Phenotypic characterization of complete CSNB in the inbred research beagle: how common is CSNB in research and companion dogs?

Oh, Annie; Loew, Ellis R.; Foster, Melanie L.; Davidson, Michael G.; English, Robert V.; Gervais, Kristen J.; Herring, Ian P.; Mowat, Freya M.

doi:10.1007/s10633-018-9653-y

Cited by 6 publications

(5 citation statements)

References 47 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…When extrapolated to the On-response it also seems likely that OFF bipolar cells make less of a contribution to the photopic a-wave of the dog than they do in primates [ 29 ]. These findings are further supported by a previously characterized canine model of complete CSNB, wherein the light-adapted On–Off ERG in affected dogs demonstrated a substantially more positive Off-response compared to control dogs (thus indicating a relatively greater role of the ON pathway in the normal canine retina) [ 46 ].…”

Section: Discussionsupporting

confidence: 69%

Characterization of scotopic and mesopic rod signaling pathways in dogs using the On–Off electroretinogram

Pasmanter

Petersen‐Jones

2022

BMC Vet Res

View full text Add to dashboard Cite

Background The On–Off, or long flash, full field electroretinogram (ERG) separates retinal responses to flash onset and offset. Depending on degree of dark-adaptation and stimulus strength the On and Off ERG can be shaped by rod and cone photoreceptors and postreceptoral cells, including ON and OFF bipolar cells. Interspecies differences have been shown, with predominantly positive Off-response in humans and other primates and a negative Off-response in rodents and dogs. However, the rod signaling pathways that contribute to these differential responses have not been characterized. In this study, we designed a long flash protocol in the dog that varied in background luminance and stimulus strength allowing for some rod components to be present to better characterize how rod pathways vary from scotopic to mesopic conditions. Results With low background light the rod a-wave remains while the b-wave is significantly reduced resulting in a predominantly negative waveform in mesopic conditions. Through modeling and subtraction of the rod-driven response, we show that rod bipolar cells saturate with dimmer backgrounds than rod photoreceptors, resulting in rod hyperpolarization contributing to a large underlying negativity with mesopic backgrounds. Conclusions Reduction in rod bipolar cell responses in mesopic conditions prior to suppression of rod photoreceptor responses may reflect the changes in signaling pathway of rod-driven responses needed to extend the range of lighting conditions over which the retina functions.

show abstract

Section: Discussionsupporting

confidence: 69%

Characterization of scotopic and mesopic rod signaling pathways in dogs using the On–Off electroretinogram

Pasmanter

Petersen‐Jones

2022

BMC Vet Res

View full text Add to dashboard Cite

show abstract

“…23 Dysfunction of the inner retina in congenital stationary night blindness and acquired conditions like central retinal artery occlusion could also present with an electronegative ERG. 24 Extra caution is advised when analysing the ERG data in the diagnosis and evaluation of interventions in patients with XLRS. 25 In greater detail, intraretinal cysts were found in the NFL from the OCT scanning of both eyes of the current case.…”

Section: Discussionmentioning

confidence: 99%

The electrophysiological features of X-linked juvenile retinoschisis in a young male: a case report

Yan

Wang

et al. 2022

J Int Med Res

View full text Add to dashboard Cite

This case report describes the detailed electrophysiological features and the corresponding relationship with the structural changes in a case of X-linked juvenile retinoschisis (XLRS). A 25-year-old male presented with a history of several years of decreased visual acuity in both eyes. The best corrected visual acuity was 20/200 in oculus dexter (OD) and 20/80 in oculus sinister. Retinoschisis was found in the macula by optical coherence tomography, which was more severe in OD. Electroretinogram revealed a similar electronegative waveform in both eyes. Visual evoked potential detected a reduced amplitude and delayed phase in P100-wave, which was worse in OD. The patient was diagnosed as XLRS and advised to undergo continuous medical observation. He was followed up for the next year, with no significant change in retinal function and structure being observed. These current findings suggest that electrophysiology permits the detailed analysis of the clinical picture of XLRS and helps to gain a deeper understanding of the pathogenesis.

show abstract

“…Mutations in LRIT3 in humans causes a form of CSNB [139]. The ERG of the affected dogs shows a lack of ON-bipolar cell function with preservation of cone OFF-bipolar contributions [140][141][142]. There is currently debate about the positioning of the LRIT3 protein which had been described as being in the synaptic tips of the bipolar cells; however, a recent publication showed that it was presynaptic, being present in photoreceptors, but bridged the synapse to influence the positioning of post-synaptic glutamate signaling proteins [143].…”

Section: Lrit3mentioning

confidence: 99%

Large Animal Models of Inherited Retinal Degenerations: A Review

Winkler

Occelli

Petersen‐Jones

2020

Cells

View full text Add to dashboard Cite

Studies utilizing large animal models of inherited retinal degeneration (IRD) have proven important in not only the development of translational therapeutic approaches, but also in improving our understanding of disease mechanisms. The dog is the predominant species utilized because spontaneous IRD is common in the canine pet population. Cats are also a source of spontaneous IRDs. Other large animal models with spontaneous IRDs include sheep, horses and non-human primates (NHP). The pig has also proven valuable due to the ease in which transgenic animals can be generated and work is ongoing to produce engineered models of other large animal species including NHP. These large animal models offer important advantages over the widely used laboratory rodent models. The globe size and dimensions more closely parallel those of humans and, most importantly, they have a retinal region of high cone density and denser photoreceptor packing for high acuity vision. Laboratory rodents lack such a retinal region and, as macular disease is a critical cause for vision loss in humans, having a comparable retinal region in model species is particularly important. This review will discuss several large animal models which have been used to study disease mechanisms relevant for the equivalent human IRD.

show abstract

Phenotypic characterization of complete CSNB in the inbred research beagle: how common is CSNB in research and companion dogs?

Cited by 6 publications

References 47 publications

Characterization of scotopic and mesopic rod signaling pathways in dogs using the On–Off electroretinogram

Characterization of scotopic and mesopic rod signaling pathways in dogs using the On–Off electroretinogram

The electrophysiological features of X-linked juvenile retinoschisis in a young male: a case report

Large Animal Models of Inherited Retinal Degenerations: A Review

Contact Info

Product

Resources

About