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