Abstract
ABSTRACTRetinitis pigmentosa (RP) is a common retinal degeneration disease caused by mutation in any gene of the photo transduction cascade and results in photoreceptor dystrophy. Over decades, several animal models have been used to address the need for the elucidation of effective therapeutics and factors regulating retinal degeneration to prohibit or renew the damaged retina. However, controversies over the immune privilege of retina during cell transplantation and the role of immune modulation during RP still remain largely uninvestigated because of the lack of suitable animal models. Here, we have developed an immunocompromised mouse model, NOD.SCID-rd1, for retinitis pigmentosa (RP) by crossing CBA/J and NOD SCID mice and selecting homozygous double mutant animals for further breeding. Characterization of the newly developed RP model indicates a similar retinal degeneration pattern as CBA/J, with a decreased apoptosis rate and rhodopsin loss. It also exhibits loss of T cells, B cells and NK cells. The NOD.SCID-rd1 model is extremely useful for allogenic and xenogenic cell-based therapeutics, as indicated by the higher cell integration capacity post transplantation. We dissect the underlying role of the immune system in the progression of RP and the effect of immune deficiency on immune privilege of the eye using comparative qPCR studies of this model and the immune-competent RP model.
Highlights
Retinal differentiation and maturation is a strictly regulated process in humans (Yang, 2004)
In our present study, we developed an immunocompromised mouse model of retinitis pigmentosa (RP) lacking in the function of Pde6b and Prkdc
Hematology All the hematological parameters of NOD.SCID-rd1 mice were comparable to CBA/J mice except total leukocytes and lymphocytes, which were significantly lower in NOD.SCID-rd1 compared with BALB/c and CBA/J (Fig. 1A)
Summary
Retinal differentiation and maturation is a strictly regulated process in humans (Yang, 2004). The retinal degeneration diseases are irreversible once the retinal cells have degenerated because the adult retina is considered to lack stem cells and the cells lost are never regenerated (Jeon et al, 1998) To address this need, the recently emerging field of regenerative medicine seems to be. Received 1 September 2016; Accepted 28 February 2017 promising where different sources of pluripotent and somatic cells are reprogrammed into a specific cell type and transplanted into the site of the defect (Bharti et al, 2014a; Ouyang et al, 2016; Siqueira, 2011) These studies remain in the initial phase, it is expected that this may open newer therapeutic options for the retinal degeneration diseases. Researchers have used a variety of retinal degeneration models according to the purpose of their study (Chang et al, 2002; Chang, 2013; Veleri et al, 2015)
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