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Active Galactosidase Beta (GLb)

GL-B; b-GAL; bGAL; ELNR1; EBP; GLB1; Lactase; Elastin Receptor 1,67kDa; Elastin Receptor 1(67kD); Acid Beta-Galactosidase

ACTIVITY TEST

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.

USAGE

Reconstitute in 10mM PBS (pH7.4) to a concentration of 0.1-1.0 mg/mL. Do not vortex.

STORAGE

Avoid repeated freeze/thaw cycles. Store at 2-8°C for one month. Aliquot and store at -80°C for 12 months.

STABILITY

The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

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Magazine Citations
DISSERTATIONEN ONLINE DER FREIEN UNIVERSITÄT BERLIN Human epidermis reconstructed from UV-B irradiated keratinocytes mimics epidermal ageing FUDISS_derivate_000000020558
European Journal of Pharmaceutics and Biopharmaceutics Increased permeability of reconstructed human epidermis from UVB-irradiated keratinocytes. pubmed:28034807
American Journal of Orthodontics and Dentofacial Orthopedics Periodontal ligament cells in adolescents and adults: Genetic level responses to orthodontic forces Pubmed: 33082075
Eur J Orthod Morphological alterations, activity, mRNA fold changes, and aging changes before and after orthodontic force application in young and adult human-derived ¡­ 34041525
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