Retinitis pigmentosa: rapid neurodegeneration is governed by slow cell death mechanisms

Halting progressive neurodegeneration in advanced retinitis pigmentosa.

Hereditary retinal degenerative diseases, such as retinitis pigmentosa (RP), are characterized by the progressive loss of rod photoreceptors followed by loss of cones. While retinal gene therapy clinical trials demonstrated temporary improvement in visual function, this approach has yet to achieve sustained functional and anatomical rescue after disease onset in patients. The lack of sustained ...

Distinct Forms of Photoreceptor Cell Death in Retinitis Pigmentosa

Apoptotic photoreceptor cell death in mouse models of retinitis pigmentosa.

Retinitis pigmentosa (RP) is a group of inherited human diseases in which photoreceptor degeneration leads to visual loss and eventually to blindness. Although mutations in the rhodopsin, peripherin, and cGMP phosphodiesterase genes have been identified in some forms of RP, it remains to be determined whether these mutations lead to photoreceptor cell death through necrotic or apoptotic mechani...

Retinitis Pigmentosa: Disease Mechanisms, Diagnosis, and Therapies

1Department of Life Sciences, Glasgow Caledonian University, Glasgow G4 0BA, UK 2School of Ophthalmology & Optometry, The Eye Hospital, Wenzhou Medical University, Wenzhou, Zhejiang 325027, China 3Department of Ophthalmology, College of Medicine, University of Florida, Gainesville, FL 32610, USA 4Sichuan Provincial Key Laboratory for Disease Gene Study, Hospital of University of Electronic Scie...

Retinitis Pigmentosa: Genes and Disease Mechanisms

Retinitis pigmentosa (RP) is a group of inherited disorders affecting 1 in 3000-7000 people and characterized by abnormalities of the photoreceptors (rods and cones) or the retinal pigment epithelium of the retina which lead to progressive visual loss. RP can be inherited in an autosomal dominant, autosomal recessive or X-linked manner. While usually limited to the eye, RP may also occur as par...