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Sensory-motor behavioral characterization of an animal model of Maroteaux-Lamy syndrome (or Mucopolysaccharidosis VI)
Maroteaux-Lamy disease, also known as mucopolysaccharidosis (MPS) VI, is an MPS disorder caused by mutations in the ARSB gene encoding for the lysosomal enzyme arysulfatase B (ARSB). Deficient ARSB activity leads to lysosomal accumulation of dermatan sulfate in a wide range of tissues and organs. Th...
Autores principales: | Saccone, Paola, Cotugno, Gabriella, Russo, Fabio, Mastrogiacomo, Rosa, Tessitore, Alessandra, Auricchio, Alberto, De Leonibus, Elvira |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3887390/ https://www.ncbi.nlm.nih.gov/pubmed/24407717 http://dx.doi.org/10.1038/srep03644 |
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