Murine mucopolysaccharidosis type VII. Characterization of a mouse with beta-glucuronidase deficiency.

We have characterized a new mutant mouse that has virtually no beta-glucuronidase activity. This biochemical defect causes a murine lysosomal storage disease that has many interesting similarities to human mucopolysaccharidosis type VII (MPS VII; Sly syndrome; beta-glucuronidase deficiency). Genetic...

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Pubblicato in:	J Clin Invest
Autori principali:	Birkenmeier, E H, Davisson, M T, Beamer, W G, Ganschow, R E, Vogler, C A, Gwynn, B, Lyford, K A, Maltais, L M, Wawrzyniak, C J
Natura:	Artigo
Lingua:	Inglês
Pubblicazione:	American Society for Clinical Investigation 1989
Soggetti:	Research Article
Accesso online:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC303816/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/2495302/ https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI114010
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