Recombinant Anti-GLB1/Beta-galactosidase antibody [EPR8250] (ab128993)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR8250] to GLB1/Beta-galactosidase
- Suitable for: Flow Cyt (Intra), WB
- Reacts with: Human
Related conjugates and formulations
Overview
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Product name
Anti-GLB1/Beta-galactosidase antibody [EPR8250]
See all GLB1/Beta-galactosidase primary antibodies -
Description
Rabbit monoclonal [EPR8250] to GLB1/Beta-galactosidase -
Host species
Rabbit -
Tested applications
Suitable for: Flow Cyt (Intra), WBmore details
Unsuitable for: ICC/IF,IHC-P or IP -
Species reactivity
Reacts with: Human
Predicted to work with: Mouse, Rat -
Immunogen
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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Positive control
- SH-SY5Y, HeLa, Human placenta, HepG2 or BxPC-3 lysate, permeabilized HeLa cells.
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General notes
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
Storage buffer
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant -
Concentration information loading...
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Purity
Protein A purified -
Clonality
Monoclonal -
Clone number
EPR8250 -
Isotype
IgG -
Research areas
Associated products
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Alternative Versions
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Isotype control
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Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab128993 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application | Abreviews | Notes |
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Flow Cyt (Intra) |
1/10 - 1/100.
ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody. |
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WB |
1/10000 - 1/50000. Predicted molecular weight: 76 kDa.
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Notes |
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Flow Cyt (Intra)
1/10 - 1/100. ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody. |
WB
1/10000 - 1/50000. Predicted molecular weight: 76 kDa. |
Target
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Function
Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans.
Isoform 2 has no beta-galactosidase catalytic activity, but plays functional roles in the formation of extracellular elastic fibers (elastogenesis) and in the development of connective tissue. Seems to be identical to the elastin-binding protein (EBP), a major component of the non-integrin cell surface receptor expressed on fibroblasts, smooth muscle cells, chondroblasts, leukocytes, and certain cancer cell types. In elastin producing cells, associates with tropoelastin intracellularly and functions as a recycling molecular chaperone which facilitates the secretions of tropoelastin and its assembly into elastic fibers. -
Involvement in disease
Defects in GLB1 are the cause of GM1-gangliosidosis type 1 (GM1G1) [MIM:230500]; also known as infantile GM1-gangliosidosis. GM1-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM1 gangliosides, glycoproteins and keratan sulfate primarily in neurons of the central nervous system. GM1G1 is characterized by onset within the first three months of life, central nervous system degeneration, coarse facial features, hepatosplenomegaly, skeletal dysmorphology reminiscent of Hurler syndrome, and rapidly progressive psychomotor deterioration. Urinary oligosaccharide levels are high. It leads to death usually between the first and second year of life.
Defects in GLB1 are the cause of GM1-gangliosidosis type 2 (GM1G2) [MIM:230600]; also known as late infantile/juvenile GM1-gangliosidosis. GM1G2 is characterized by onset between ages 1 and 5. The main symptom is locomotor ataxia, ultimately leading to a state of decerebration with epileptic seizures. Patients do not display the skeletal changes associated with the infantile form, but they nonetheless excrete elevated amounts of beta-linked galactose-terminal oligosaccharides. Inheritance is autosomal recessive.
Defects in GLB1 are the cause of GM1-gangliosidosis type 3 (GM1G3) [MIM:230650]; also known as adult or chronic GM1-gangliosidosis. GM1G3 is characterized by a variable phenotype. Patients show mild skeletal abnormalities, dysarthria, gait disturbance, dystonia and visual impairment. Visceromegaly is absent. Intellectual deficit can initially be mild or absent but progresses over time. Inheritance is autosomal recessive.
Defects in GLB1 are the cause of mucopolysaccharidosis type 4B (MPS4B) [MIM:253010]; also known as Morquio syndrome B. MPS4B is a form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life. -
Sequence similarities
Belongs to the glycosyl hydrolase 35 family. -
Cellular localization
Lysosome and Cytoplasm > perinuclear region. Localized to the perinuclear area of the cytoplasm but not to lysosomes. - Information by UniProt
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Database links
- Entrez Gene: 2720 Human
- Entrez Gene: 12091 Mouse
- Entrez Gene: 316033 Rat
- Omim: 611458 Human
- SwissProt: P16278 Human
- SwissProt: P23780 Mouse
- Unigene: 443031 Human
- Unigene: 290516 Mouse
see all -
Alternative names
- Acid beta galactosidase antibody
- Acid beta-galactosidase antibody
- Beta galactosidase 1 antibody
see all
Images
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All lanes : Anti-GLB1/Beta-galactosidase antibody [EPR8250] (ab128993) at 1/10000 dilution
Lane 1 : SH-SY5Y cell lysate
Lane 2 : HeLa cell lysate
Lane 3 : Human placenta cell lysate
Lane 4 : HepG2 cell lysate
Lane 5 : BxPC-3 cell lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : HRP labelled goat
anti-rabbit at 1/2000 dilution
Predicted band size: 76 kDa -
Intracellular flow cytometric analysis of permeabilized HeLa cells using ab128933 (red) or a rabbit IgG (negative) (green).
Datasheets and documents
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SDS download
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Datasheet download
References (4)
ab128993 has been referenced in 4 publications.
- Cui Y et al. Retromer has a selective function in cargo sorting via endosome transport carriers. J Cell Biol 218:615-631 (2019). PubMed: 30559172
- Mortensen ACL et al. The Stapled Peptide PM2 Stabilizes p53 Levels and Radiosensitizes Wild-Type p53 Cancer Cells. Front Oncol 9:923 (2019). PubMed: 31616635
- Yan Y et al. Laminin a4 overexpression in the anterior lens capsule may contribute to the senescence of human lens epithelial cells in age-related cataract. Aging (Albany NY) 11:2699-2723 (2019). PubMed: 31076560
- Xing J et al. Hypoxia induces senescence of bone marrow mesenchymal stem cells via altered gut microbiota. Nat Commun 9:2020 (2018). PubMed: 29789585