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

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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Bibliographic Details
Main Authors: Sean C. Devanney, Joseph M. Gibney, Colleen G. Le Prell, Thomas J. Wronski, J.I. Aguirre, Issam Mcdoom, Coy D. Heldermon
Format: Article
Language:English
Published: Elsevier 2021-06-01
Series:Molecular Genetics and Metabolism Reports
Subjects:
Mouse
Mucopolysaccharidosis VII
Sly syndrome (MPS VII)
Survival
Bone
Retina
Online Access:http://www.sciencedirect.com/science/article/pii/S2214426921000215

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