Mechanisms of neurodegeneration in mucopolysaccharidoses II and IIIB: analysis of human brain tissue

@article{Hamano2007MechanismsON,
  title={Mechanisms of neurodegeneration in mucopolysaccharidoses II and IIIB: analysis of human brain tissue},
  author={Kimiko Hamano and Masaharu Hayashi and Kei Shioda and Ryo Fukatsu and Shuki Mizutani},
  journal={Acta Neuropathologica},
  year={2007},
  volume={115},
  pages={547-559}
}
Mucopolysaccharidoses (MPS) are inherited disorders caused by the deficiency of lysosomal enzymes. Sanfilippo syndrome (MPS III) and Hunter syndrome (MPS II) are characterized by severe and mild neurological disorders, respectively, in which the neurodegenerative mechanisms remain to be clarified. We immunohistochemically examined the involvement of tauopathy/synucleinopathy, cell death and oxidative damage in the brains of three cases each of MPS IIIB and MPS II and age-matched controls. In… CONTINUE READING