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Transgene produces massive overexpression of human β-glucuronidase in mice, lysosomal storage of enzyme, and strain-dependent tumors

β-Glucuronidase (GUSB) is a lysosomal enzyme important in the normal step-wise degradation of glycosaminoglycans. Deficiency of GUSB causes the lysosomal storage disease mucopolysaccharidosis VII (MPS VII, Sly disease). Affected patients have widespread progressive accumulation of β-glucuronide-cont...

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Udgivet i:Proc Natl Acad Sci U S A
Principais autores: Vogler, Carole, Galvin, Nancy, Levy, Beth, Grubb, Jeffery, Jiang, Jinxing, Zhou, Xiao Yan, Sly, William S.
Format: Artigo
Sprog:Inglês
Udgivet: National Academy of Sciences 2003
Fag:
Biological Sciences
Online adgang:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC151398/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/12591953/
https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.0437941100
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