Retinal Degeneration in Mice Deficient in the Lysosomal Membrane Protein CLN7 | Zendy

Wanda Jankowiak | Zendy; Laura Brandenstein | Zendy; Simon Dulz | Zendy; Christian Hagel | Zendy; Stephan Storch | Zendy; Udo Bartsch | Zendy

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Retinal Degeneration in Mice Deficient in the Lysosomal Membrane Protein CLN7

Author(s) -

Wanda Jankowiak,

Laura Brandenstein,

Simon Dulz,

Christian Hagel,

Stephan Storch,

Udo Bartsch

Publication year - 2016

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.16-20158

Subject(s) - retinal degeneration , astrogliosis , neurodegeneration , biology , neuronal ceroid lipofuscinosis , retina , microbiology and biotechnology , batten disease , pathology , retinal , immunohistochemistry , lipofuscin , neuroscience , biochemistry , immunology , medicine , disease , central nervous system , gene

Neuronal ceroid lipofuscinoses comprise a genetically heterogeneous group of mainly childhood-onset neurodegenerative lysosomal storage disorders. Progressive loss of vision is among the typical clinical symptoms of these fatal disorders. Here, we performed a detailed analysis of retinal degeneration in mice deficient in the lysosomal membrane protein CLN7, a novel animal model of CLN7 disease.

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