The beta-glucuronidase intracisternal A particle insertion model results in similar overall MPSVII phenotype as the single base deletion model when on the same C57BL/6J mouse background

Two unique gene mutations in the enzyme beta-glucuronidase (GUSB) that result in the lysosomal storage disease Mucopolysaccharidosis (MPS) type VII had previously been reported to have differing disease phenotype severities when compared on differing mouse strains. The MPSVII mouse has proven to be...

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Detalles Bibliográficos
Autores principales: Devanney, Sean C., Gibney, Joseph M., Le Prell, Colleen G., Wronski, Thomas J., Aguirre, J.I., Mcdoom, Issam, Heldermon, Coy D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
Materias:
Research Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7873470/
https://www.ncbi.nlm.nih.gov/pubmed/33604242
http://dx.doi.org/10.1016/j.ymgmr.2021.100727

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