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...

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Bibliografiske detaljer
Main Authors: Saccone, Paola, Cotugno, Gabriella, Russo, Fabio, Mastrogiacomo, Rosa, Tessitore, Alessandra, Auricchio, Alberto, De Leonibus, Elvira
Format: Artigo
Sprog:Inglês
Udgivet: Nature Publishing Group 2014
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3887390/
https://ncbi.nlm.nih.gov/pubmed/24407717
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep03644
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