Recombinant Human GLB1 Protein: Properties & Applications

Recombinant Human GLB1 Protein is a lysosomal acid beta-galactosidase produced recombinantly to support research into lysosomal storage disorders, enzyme kinetics, and glycan catabolism.

Product SKU

rp367958

Protein Name

Recombinant Human GLB1 (Beta-Galactosidase-1)

Gene Name

GLB1

Protein Origin

Human

Biological Function

Lysosomal acid beta-galactosidase; hydrolyzes terminal beta-galactose residues from gangliosides, glycoproteins, and glycosaminoglycans

Disease Relevance

GM1 gangliosidosis and Morquio B syndrome (MPS IVB)

Reference Number

absea#PP-11418

Biological Background of GLB1

GLB1 encodes lysosomal acid beta-galactosidase (EC 3.2.1.23), an enzyme responsible for the hydrolysis of terminal beta-D-galactose residues from a range of substrates including GM1 gangliosides, glycoproteins, and keratan sulfate. The enzyme is ubiquitously expressed and plays a critical role in the stepwise degradation of glycoconjugates within the lysosomal compartment.

Loss-of-function mutations in the GLB1 gene lead to two distinct lysosomal storage disorders: GM1 gangliosidosis, characterized by progressive neurodegeneration due to accumulation of GM1 ganglioside, and Morquio B syndrome (mucopolysaccharidosis type IVB), in which keratan sulfate accumulates primarily in skeletal tissues. The severity and clinical phenotype depend on the specific mutation and the level of residual enzyme activity.

Beyond its role in substrate catabolism, GLB1 has been implicated in cellular senescence pathways. Research indicates that GLB1 activity is used as a biomarker for senescent cells (senescence-associated beta-galactosidase, SA-β-Gal activity), making it a molecule of broad research interest extending beyond lysosomal storage disease into aging biology and oncology.

Protein Properties and Production Specifications

Recombinant Human GLB1 Protein (SKU: rp367958) is produced using recombinant DNA technology to ensure consistent batch-to-batch quality and biological activity. The protein is derived from the human GLB1 gene sequence and is intended for in vitro research use only.

Key production and quality parameters typically evaluated for recombinant lysosomal enzymes of this class include:

• Expression system: Mammalian or insect cell expression systems are commonly used to ensure proper post-translational modifications, including glycosylation critical for lysosomal targeting and enzymatic activity. Researchers should consult the product datasheet for the specific system used for rp367958.
• Purity: High-purity preparations (typically >90% by SDS-PAGE) are standard for recombinant proteins intended for biochemical and cell-based assays.
• Biological activity: Enzyme activity is confirmed using appropriate fluorogenic or chromogenic substrates (e.g., 4-methylumbelliferyl-beta-D-galactopyranoside, 4-MU-beta-Gal) under defined assay conditions.
• Formulation: Typically supplied in a buffered solution with stabilizing excipients; refer to the product datasheet for exact formulation details.
• Storage: Generally recommended at -20°C or -80°C; avoid repeated freeze-thaw cycles to preserve activity.

For precise molecular weight, tag information, endotoxin levels, and lot-specific activity data, researchers should refer to the Certificate of Analysis (CoA) supplied with each lot of rp367958.

Research Applications

Recombinant Human GLB1 Protein supports a broad range of in vitro research applications:

• Enzyme activity assays: GLB1 recombinant protein serves as a positive control or reference enzyme in fluorometric and colorimetric assays measuring beta-galactosidase activity, including diagnostic and mechanistic studies of GM1 gangliosidosis and Morquio B syndrome.
• Drug discovery and enzyme replacement therapy (ERT) research: Recombinant GLB1 is used to evaluate potential small-molecule chaperones, pharmacological chaperones, and substrate reduction therapy candidates targeting lysosomal storage disorders.
• Senescence research: Because SA-β-Gal activity is a widely used biomarker of cellular senescence, recombinant GLB1 can serve as a standard in assays designed to quantify or characterize senescent cell populations in vitro.
• Structural and biochemical studies: The purified recombinant protein enables kinetic characterization (Km, Vmax, inhibitor constants) and structural analyses of the GLB1 enzyme under controlled conditions.
• Antibody validation and immunoassay development: Recombinant GLB1 is useful as an antigen for validating anti-GLB1 antibodies in Western blotting, ELISA, and immunoprecipitation applications.

These applications make rp367958 a versatile reagent for laboratories working in lysosomal biology, rare disease research, aging, and related translational research areas.

Handling, Storage, and Quality Considerations

Proper handling of Recombinant Human GLB1 Protein is essential to maintain its biological activity and experimental reproducibility:

• Reconstitution: If supplied lyophilized, reconstitute according to the datasheet instructions using the recommended diluent (typically sterile water or a low-salt buffer). Allow the vial to equilibrate to room temperature before opening.
• Aliquoting: Prepare single-use aliquots immediately after reconstitution to minimize activity loss from repeated freeze-thaw cycles.
• Storage temperature: Store at -20°C for short-term or -80°C for long-term storage. Do not store at 4°C for extended periods, as this may lead to aggregation or activity loss.
• Carrier protein: If the product is supplied without carrier protein, consider adding BSA (0.1% final concentration) to diluted working solutions to stabilize activity at low protein concentrations.
• Endotoxin levels: For cell-based assays, verify endotoxin levels in the CoA to avoid confounding inflammatory responses.
• Quality control: Each lot of rp367958 should be accompanied by a CoA confirming purity, identity, and activity. Researchers should retain CoA documents for experimental records and regulatory compliance.

Frequently asked questions

What is the biological function of the GLB1 protein?

GLB1 encodes lysosomal acid beta-galactosidase (EC 3.2.1.23), which hydrolyzes terminal beta-D-galactose residues from glycoconjugates including GM1 gangliosides, glycoproteins, and keratan sulfate. Deficiency of this enzyme causes GM1 gangliosidosis and Morquio B syndrome. GLB1 activity is also widely used as a biomarker (SA-β-Gal) for cellular senescence.

What research applications is Recombinant Human GLB1 Protein (rp367958) suitable for?

rp367958 is suitable for enzyme activity assays, drug discovery research targeting lysosomal storage disorders, cellular senescence studies, structural and kinetic biochemical analyses, and antibody validation or immunoassay development. It is intended for in vitro research use only.

How should Recombinant Human GLB1 Protein be stored to maintain activity?

The protein should be stored at -20°C for short-term use or at -80°C for long-term storage. Repeated freeze-thaw cycles should be avoided by preparing single-use aliquots. Refer to the product datasheet and Certificate of Analysis for lot-specific storage recommendations.

What diseases are associated with GLB1 deficiency?

Deficiency of lysosomal acid beta-galactosidase encoded by GLB1 leads to two lysosomal storage disorders: GM1 gangliosidosis, which causes progressive neurodegeneration due to GM1 ganglioside accumulation, and Morquio B syndrome (mucopolysaccharidosis type IVB), characterized by skeletal dysplasia due to keratan sulfate accumulation.

How is beta-galactosidase activity typically measured in research assays?

Beta-galactosidase activity is commonly measured using fluorogenic substrates such as 4-methylumbelliferyl-beta-D-galactopyranoside (4-MU-beta-Gal), which releases a fluorescent product upon enzymatic cleavage. Colorimetric substrates such as ortho-nitrophenyl-beta-D-galactopyranoside (ONPG) are also used. Assays are typically performed at acidic pH conditions to reflect lysosomal enzyme activity.

References

1. Suzuki Y, Oshima A, Nanba E. Beta-galactosidase deficiency (beta-galactosidosis): GM1 gangliosidosis and Morquio B disease. In: Scriver CR et al., eds. The Metabolic and Molecular Bases of Inherited Disease. 8th ed. McGraw-Hill; 2001.
2. Dimri GP, Lee X, Basile G, et al. A biomarker that identifies senescent human cells in culture and in aging skin in vivo. Proc Natl Acad Sci USA. 1995;92(20):9363-9367. doi:10.1073/pnas.92.20.9363
3. Morrone A, et al. Molecular basis of glycogen storage disease type IVB (Morquio B): characterization of beta-galactosidase gene mutations. Hum Mutat. 2000.

Products are supplied for research and development use only. Not for use in humans, animals, diagnosis, or therapy.