Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF

R&D Systems, part of Bio-Techne | Catalog # 6464-GH

R&D Systems, part of Bio-Techne
Catalog #
Availability
Size / Price
Qty
6464-GH-020
Print Quote
Bulk Order
100% Guarantee

Key Product Details

Source

CHO

Accession #

AAA51819

Conjugate

Unconjugated

Applications

Enzyme Activity

View all beta-Galactosidase-1/GLB1 Proteins and Enzymes »

Product Specifications

Source

Chinese Hamster Ovary cell line, CHO-derived human beta-Galactosidase-1/GLB1 protein
Met1-Val677, with a C-terminal 6-His tag

Purity

>95%, by SDS-PAGE visualized with Silver Staining and quantitative densitometry by Coomassie® Blue Staining.

Endotoxin Level

<1.0 EU per 1 μg of the protein by the LAL method.

N-terminal Sequence Analysis

Leu24

Predicted Molecular Mass

74 kDa

SDS-PAGE

70-95 kDa, reducing conditions

Activity

Measured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl-beta -D-galactopyranoside.
The specific activity is >1,800 pmol/min/μg, as measured under the described conditions.

Scientific Data Images for Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF

Recombinant Human beta-Galactosidase-1/GLB1 Protein Enzyme Activity Diagram.

Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF (Catalog # 6464-GH) removes terminal galactose residues from various glycolipids, glycoproteins, and glycosaminoglycans.

Recombinant Human beta‑Galactosidase‑1/GLB1 Protein Enzyme Activity.

Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF (Catalog # 6464-GH) is measured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl-beta -D-galactopyranoside.

Recombinant Human beta‑Galactosidase‑1/GLB1 Protein SDS-PAGE.

2 μg/lane of Recombinant Human beta‑Galactosidase‑1/GLB1 Protein (Catalog # 6464-GH) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 70-95 kDa.

Formulation, Preparation and Storage

6464-GH
Formulation Supplied as a 0.2 μm filtered solution in Tris and NaCl.
Shipping The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 6 months from date of receipt, -20 to -70 °C as supplied.
  • 3 months, -20 to -70 °C under sterile conditions after opening.

Background: beta-Galactosidase-1/GLB1

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) (1, 2, 3). In GM1 gangliosidosis, GM1 ganglioside accumulates in the neurons of the central nervous system, because of the deficiency (0±3% of normal) 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 (4). More than 100 mutations have been identified for GLB1, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases (5). In lysosome, the mature beta-galactosidase protein associates with cathepsin A and neuraminidase 1 to form the lysosomal multienzyme complex (6). An alternative splicing at the RNA level of GLB1 results a catalytically inactive beta-galactosidase (also called elastin-binding protein) that plays an important role in vascular development (7).

References

  1. Hofer, D. et al. (2009) Hum. Mutat. 30:1214.
  2. Brunetti-Pierri N, and Scaglia F. (2008) Mol. Genet. Metab. 94:391.
  3. Santamaria, R. et al. (2007) J. Lipid Res. 48:2275.
  4. Prat, C. (2008) Joint Bone Spine, 75:495.
  5. Zhang, S. et al. (2000) Biochem. J. 348:621.
  6. Pshezhetsky, A.V. and Ashmarina, M. (2001) Prog. Nucleic Acid Res. Mol. Biol. 69:81.
  7. Salvatore P, et al. (1998) J. Biol. Chem. 273:6319.

Alternate Names

betaGalactosidase1, EBP, ELNR1, GLB1, Lactase

Entrez Gene IDs

2720 (Human)

Gene Symbol

GLB1

UniProt

AAA51819

Additional beta-Galactosidase-1/GLB1 Products

Product Documents for Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF

Product Datasheets

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Note: Certificate of Analysis not available for kit components.

Download SDS

Product Specific Notices for Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF

For research use only

Privacy and Cookie Policy
Site Map
© Copyright 2024 Bio-Techne. All Rights Reserved.