Degeneration of Photoreceptor Cells in Arylsulfatase G-Deficient Mice | Zendy

Katharina Kruszewski | Zendy; Renate LüllmannRauch | Zendy; Thomas Dierks | Zendy; Udo Bartsch | Zendy; Markus Daμμe | Zendy

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Degeneration of Photoreceptor Cells in Arylsulfatase G-Deficient Mice

Author(s) -

Katharina Kruszewski,

Renate LüllmannRauch,

Thomas Dierks,

Udo Bartsch,

Markus Daμμe

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.15-17645

Subject(s) - retinal degeneration , retina , astrogliosis , mucopolysaccharidosis , knockout mouse , biology , photoreceptor cell , lysosomal storage disease , neuronal ceroid lipofuscinosis , retinal , electroretinography , gliosis , microbiology and biotechnology , microglia , western blot , pathology , retinitis pigmentosa , endocrinology , neuroscience , immunology , medicine , biochemistry , central nervous system , enzyme , gene , inflammation

Retinal degeneration is a common feature of several lysosomal storage disorders, including the mucopolysaccharidoses, a group of metabolic disorders that is characterized by widespread accumulation of glycosaminoglycans due to lysosomal enzyme dysfunction. We used a new mouse model of mucopolysaccharidosis IIIE to study the effect of Arylsulfatase G (ARSG) deficiency on retina integrity.

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