Cargando…
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...
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 |
Ejemplares similares
-
An Uncommon Presentation of Mucopolysaccharidosis Type IIIb
por: REZAYI, Alireza, et al.
Publicado: (2019) -
Trehalose reduces retinal degeneration, neuroinflammation and storage burden caused by a lysosomal hydrolase deficiency
por: Lotfi, Parisa, et al.
Publicado: (2018) -
A model of mucopolysaccharidosis type IIIB in pigs
por: Yang, Qiang, et al.
Publicado: (2018) -
Proteomic Analysis of Mucopolysaccharidosis IIIB Mouse Brain
por: De Pasquale, Valeria, et al.
Publicado: (2020) -
Activation of Stress Kinases in the Brain of Mucopolysaccharidosis IIIB mice
por: Cecere, Francesca, et al.
Publicado: (2011)