Failure of Autophagy–Lysosomal Pathways in Rod Photoreceptors Causes the Early Retinal Degeneration Phenotype Observed inCln6nclfMice | Zendy

Philipp von Eisenhart-Rothe | Zendy; Alexandra Grubman | Zendy; Ursula Greferath | Zendy; Linda J. Fothergill | Zendy; Andrew I. Jobling | Zendy; Joanna A. Phipps | Zendy; Anthony R. White | Zendy; Erica L. Fletcher | Zendy; Kirstan A. Vessey | Zendy

Open Access

Failure of Autophagy–Lysosomal Pathways in Rod Photoreceptors Causes the Early Retinal Degeneration Phenotype Observed inCln6^nclfMice

Author(s) -

Philipp von Eisenhart-Rothe,

Alexandra Grubman,

Ursula Greferath,

Linda J. Fothergill,

Andrew I. Jobling,

Joanna A. Phipps,

Anthony R. White,

Erica L. Fletcher,

Kirstan A. Vessey

Publication year - 2018

Publication title -

investigative ophthalmology and visual science

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.935

H-Index - 218

eISSN - 1552-5783

pISSN - 0146-0404

DOI - 10.1167/iovs.18-24757

Subject(s) - retinal degeneration , lipofuscin , neuronal ceroid lipofuscinosis , retinal , autophagy , microbiology and biotechnology , retinitis pigmentosa , biology , batten disease , retina , lysosome , electroretinography , mitochondrion , retinal pigment epithelium , degeneration (medical) , pathology , neuroscience , biochemistry , apoptosis , medicine , gene , enzyme

Vision loss caused by photoreceptor death represents one of the first symptoms in neuronal ceroid lipofuscinosis, a condition characterized by accumulation of intracellular waste. Cln6nclf mice have a naturally occurring mutation in ceroid-lipofuscinosis neuronal (CLN) protein 6 and are a model of this disorder. In order to identify the effect intracellular waste (lipofuscin) accumulation plays in driving retinal degeneration, the time course of degeneration was carefully characterized functionally using the electroretinogram and structurally using histology.

The content you want is available to Zendy users.

Already have an account? Click here to sign in.

Having issues? You can contact us here

Accelerating Research