Expression of Bcl-2 Protects against Photoreceptor Degeneration inretinal degeneration slow(rds) Mice

Authors:
Open Access
Publisher website
Google Scholar
Abstract
Theretinal degeneration sloworrdsgene encodes rds/peripherin, an integral membrane glycoprotein in the outer segments of rod and cone photoreceptors. Mice homozygous for a null mutation inrdsfail to develop outer segments and undergo subsequent degeneration of photoreceptors by the apoptotic pathway. Mutations in the humanRDSgene are responsible for several forms of inherited blindness including autosomal-dominant retinitis pigmentosa and macular degeneration. Here, we examined the effects of ectopic Bcl-2 expression in transgenic photoreceptors on the rate of retinal degeneration inrdsmutant mice. We observed an approximately twofold preservation of photoreceptors compared with nontransgenicrdsmutant mice at 3 months. Immunoblot analysis showed similar levels of Bcl-2 in 2-, 3-, and 4-week-old transgenic mice. Expression of Bcl-2 in therdsmouse did not lead to outer segment formation and did not induce cell death. These results suggest that Bcl-2 expression may be an effective therapeutic strategy in humans with mutations inRDSor other genes that affect the integrity of photoreceptor outer segments.

This publication has 27 references indexed in Scilit: