GLB1 is a lysosomal beta -galactosidase that hydrolyzes the terminal beta -galactose from ganglioside and keratan sulfate. Defects in this gene are the causes of lysosomal storage diseases for GM1-gangliosidosis and Morquio B syndrome (also known as mucopolysaccharidosis IVB). In GM1 gangliosidosis, GM1 ganglioside accumulates in the neurons of the central nervous system, because of the deficiency of lysosomal beta -galactosidase activity. GM1 gangliosidosis demonstrates varying degrees of clinical severity but is invariably fatal, and children with the most common and severe form of GM1 gangliosidosis usually die within 3 years of birth. Morquio B syndrome patients are neurologically normal, but display severe skeletal dysostosis multiplex because of an accumulation of keratan sulfate. The activity assay of GLB1 was measured by its ability to cleave a peptide substrate, 4-Methylumbelliferyl-beta -D-galactopyranoside. The reaction was performed in 50 mM Sodium Citrate, pH 3.5 ( Assay Buffer), ainitiated by addition 50 μL of 1.5 ug/ml uPA (diluted by Assay Buffer) to 50 μL of 1.2 mM Substrate. Read at excitation and emission wavelengths of 365 nm and 445 nm (top read), respectively, in kinetic mode for 5 minutes. The specific activity of recombinant mouse GLB1 is >17000 pmol/min/μg.