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Electrophysiological and Histological Characterization of Rod-Cone Retinal Degeneration and Microglia Activation in a Mouse Model of Mucopolysaccharidosis Type IIIB

Sanfilippo syndrome Type B or Mucopolysaccharidosis IIIB (MPS IIIB) is a neurodegenerative autosomal recessive lysosomal storage disorder in which patients suffer severe vision loss from associated retinopathy. Here we sought to study the underlying retinal functional and morphological changes assoc...

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Detalles Bibliográficos
Autores principales: Tse, Dennis Y., Lotfi, Parisa, Simons, David L., Sardiello, Marco, Wu, Samuel M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4660851/
https://www.ncbi.nlm.nih.gov/pubmed/26607664
http://dx.doi.org/10.1038/srep17143