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Lysosomal Accumulation of SCMAS (Subunit c of Mitochondrial ATP Synthase) in Neurons of the Mouse Model of Mucopolysaccharidosis III B

The neurodegenerative disease MPS III B (Sanfilippo syndrome type B) is caused by mutations in the gene encoding the lysosomal enzyme α-N-acetylglucosaminidase, with a resulting block in heparan sulfate degradation. A mouse model with disruption of the Naglu gene allows detailed study of brain patho...

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Autors principals: Ryazantsev, Sergey, Yu, Wei-Hong, Zhao, Hui-Zhi, Neufeld, Elizabeth F., Ohmi, Kazuhiro
Format: Artigo
Idioma:Inglês
Publicat: 2006
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC1892176/
https://ncbi.nlm.nih.gov/pubmed/17185018
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2006.11.006
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