Key features and details
- Rabbit polyclonal to GLB1/Beta-galactosidase
- Suitable for: IHC-P
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
Product nameAnti-GLB1/Beta-galactosidase antibody
See all GLB1/Beta-galactosidase primary antibodies
DescriptionRabbit polyclonal to GLB1/Beta-galactosidase
Tested applicationsSuitable for: IHC-Pmore details
Species reactivityReacts with: Mouse, Rat, Human
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.09% Sodium azide
Constituents: 50% Glycerol, 0.01% BSA
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab203749 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/100 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
Use at 1/50 - 1/200 with fluorescent detection methods.
FunctionCleaves 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 diseaseDefects 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 similaritiesBelongs to the glycosyl hydrolase 35 family.
Cellular localizationLysosome and Cytoplasm > perinuclear region. Localized to the perinuclear area of the cytoplasm but not to lysosomes.
- Information by UniProt
- Acid beta galactosidase antibody
- Acid beta-galactosidase antibody
- Beta galactosidase 1 antibody
Immunohistochemical analysis of formalin-fixed and paraffin embedded mouse embryo tissue labeling GLB1/Beta-galactosidase using ab203749 at 1/200 dilution, followed by secondary detection and DAB staining.
Immunohistochemical analysis of formalin-fixed and paraffin embedded mouse liver tissue labeling GLB1/Beta-galactosidase using ab203749 at 1/200 dilution, followed by secondary detection and DAB staining.
ab203749 has been referenced in 5 publications.
- Che H et al. p16 deficiency attenuates intervertebral disc degeneration by adjusting oxidative stress and nucleus pulposus cell cycle. Elife 9:N/A (2020). PubMed: 32125276
- Xie X et al. Overexpressed vascular endothelial growth factor in adipose derived stem cells attenuates fibroblasts and skin injuries by ultraviolet radiation. Biosci Rep 39:N/A (2019). PubMed: 31266813
- Kumar S et al. Intestinal stem cells acquire premature senescence and senescence associated secretory phenotype concurrent with persistent DNA damage after heavy ion radiation in mice. Aging (Albany NY) 11:4145-4158 (2019). PubMed: 31239406
- Patel P et al. Dual Inhibition of CDK4 and CDK2 via Targeting p27 Tyrosine Phosphorylation Induces a Potent and Durable Response in Breast Cancer Cells. Mol Cancer Res 16:361-377 (2018). PubMed: 29330290
- Hall BM et al. Aging of mice is associated with p16(Ink4a)- and ß-galactosidase-positive macrophage accumulation that can be induced in young mice by senescent cells. Aging (Albany NY) 8:1294-315 (2016). IHC-Fr ; Mouse . PubMed: 27391570