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Murine β-galactosidase stability is not dependent...
Journal article

Murine β-galactosidase stability is not dependent on temperature or protective protein/cathepsin A

Abstract

GM1 gangliosidosis, a neurodegenerative disorder, and Morquio B disease, a skeletal disorder, are lysosomal storage disorders caused by inherited defects in the enzyme β-galactosidase (GLB1; EC 3.1.2.23; MIM #611458). Enzyme replacement therapy (ERT), a standard of care for a number of non-neuronopathic lysosomal storage disorders, is not yet available for GLB1 deficiency. Although functionally active recombinant human and feline GLB1 …

Authors

Lambourne MD; Potter MA

Journal

Molecular Genetics and Metabolism, Vol. 104, No. 4, pp. 620–626

Publisher

Elsevier

Publication Date

December 2011

DOI

10.1016/j.ymgme.2011.09.016

ISSN

1096-7192