Ocular Effects of Prion Disease

Article: Scientists identify locations of early prion protein deposition in retina
Source: National Institutes of Health
Published: January 29, 2021
Article: A Closer Look at Prion Disease and Its Effect on the Eye
Source: Genetic Engineering & Biotechnology News
Published: February 1, 2021

PrPSc present at the location of the cilium
(arrow) between the inner and outer segments

Prion diseases are neurodegenerative diseases due to misfolded proteins and usually affect the brain. Because the retina is an extension of the brain, many neurodegenerative diseases also have ocular manifestations. In this study, scientists at the NIH's National Institute of Allergy and Infectious Diseases investigated the mechanism of prion diseases on the eye's photoreceptors using a mouse model of scrapie, a prion disease common to sheep and goats. Previously, the researchers found that prion-induced retinal damage in mice and human photoreceptors resembled that of retinitis pigmentosa. In the current study, they further isolated the location of that damage to the cilia and ribbon synapses of rod photoreceptors. By interfering with transport and critical synapses, prion aggregates may provide an early mechanism of damage to the photoreceptors. The findings in this study provide a new level of detail to the association between prion disease and retinal damage.

My rating of this study: ⭐⭐

Striebel JF, Race B, Leung JM, et al. "Prion-induced photoreceptor degeneration begins with misfolded prion protein accumulation in cones at two distinct sites: cilia and ribbon synapses." Acta Neuropathologica Communications.  9(17). 29 January 2021. https://doi.org/10.1186/s40478-021-01120-x