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Secondary Storage of Dermatan Sulfate in Sanfilippo Disease
Mucopolysaccharidoses are a group of genetically inherited disorders that result from the defective activity of lysosomal enzymes involved in glycosaminoglycan catabolism, causing their intralysosomal accumulation. Sanfilippo disease describes a subset of mucopolysaccharidoses resulting from defects...
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| Main Authors: | , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
American Society for Biochemistry and Molecular Biology
2011
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3044951/ https://ncbi.nlm.nih.gov/pubmed/21193389 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.192062 |
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