Retinal Degeneration in MPS-IIIA Mouse Model

Retinal Degeneration in MPS-IIIA Mouse Model

Mucopolysaccharidosis type IIIA (MPS-IIIA, Sanfilippo A) is one of the most severe lysosomal storage disorder (LSD) caused by the inherited deficiency of sulfamidase, a lysosomal sulfatase enzyme involved in the stepwise degradation of heparan sulfates (HS). MPS-IIIA patients show multisystemic prob...

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Bibliographic Details
Main Authors: Daniela Intartaglia, Giuliana Giamundo, Elena Marrocco, Veronica Maffia, Francesco Giuseppe Salierno, Edoardo Nusco, Alessandro Fraldi, Ivan Conte, Nicolina Cristina Sorrentino
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-03-01
Series:Frontiers in Cell and Developmental Biology
Subjects:
retina
MPS-IIIA
lysosomal storage disease
autophagy
Sanfilippo A syndrome
Online Access:https://www.frontiersin.org/article/10.3389/fcell.2020.00132/full

Internet

https://www.frontiersin.org/article/10.3389/fcell.2020.00132/full

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