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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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Enregistré dans:
Détails bibliographiques
Publié dans:Sci Rep
Auteurs principaux: Tse, Dennis Y., Lotfi, Parisa, Simons, David L., Sardiello, Marco, Wu, Samuel M.
Format: Artigo
Langue:Inglês
Publié: Nature Publishing Group 2015
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC4660851/
https://ncbi.nlm.nih.gov/pubmed/26607664
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep17143
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