Mucopolysaccharidosis IIIB: oxidative damage and cytotoxic cell involvement in the neuronal pathogenesis.

@article{Villani2009MucopolysaccharidosisIO,
  title={Mucopolysaccharidosis IIIB: oxidative damage and cytotoxic cell involvement in the neuronal pathogenesis.},
  author={Guglielmo Rd Villani and Carmela Di Domenico and Annapaola Musella and Francesca Cecere and Daniele Di Napoli and Paola di Natale},
  journal={Brain research},
  year={2009},
  volume={1279},
  pages={99-108}
}
Sanfilippo B syndrome (Mucopolysaccharidosis IIIB, MPS IIIB) is a lysosomal storage disease due to mutations in the gene encoding alpha-N-acetylglucosaminidase and is characterized by a severe neurological disorder. Although several studies have been reported for the murine model of the disease, the molecular basis and the sequence of events leading to neurodegeneration remain to be clarified. We previously suggested the possible involvement of the reactive oxygen species in the disease… CONTINUE READING
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