• Product nameAnti-GLB1 antibody
    See all GLB1 primary antibodies
  • Description
    Mouse polyclonal to GLB1
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Full length Human GLB1 protein (AAH07493.1).

  • Positive control
    • Human pancreas tissue lysate and GLB1 transfected 293T cell lysate.


Associated products


Our Abpromise guarantee covers the use of ab89084 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
WB 1/500 - 1/1000. Predicted molecular weight: 76 kDa.


  • 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
  • Database links
  • Alternative names
    • Acid beta galactosidase antibody
    • Acid beta-galactosidase antibody
    • Beta galactosidase 1 antibody
    • Beta galactosidase antibody
    • Beta-galactosidase antibody
    • BGAL_HUMAN antibody
    • EBP antibody
    • EBP, included antibody
    • Elastin receptor 1 (67kD) antibody
    • Elastin receptor 1 67kDa antibody
    • Elastin receptor 1 antibody
    • Elastin receptor 1, included antibody
    • Elastin-binding protein, included antibody
    • ELNR1 antibody
    • Galactosidase beta 1 antibody
    • GLB 1 antibody
    • GLB1 antibody
    • Lactase antibody
    • MPS4B antibody
    • S-GAL, included antibody
    see all

Anti-GLB1 antibody images

  • Anti-GLB1 antibody (ab89084) at 1/500 dilution + human pancreas tissue lysate at 50 µg

    Predicted band size : 76 kDa
    Observed band size : 73 kDa (why is the actual band size different from the predicted?)
  • All lanes : Anti-GLB1 antibody (ab89084) at 1/500 dilution

    Lane 1 : GLB1 transfected 293T cell lysate
    Lane 2 : Non transfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.

    Predicted band size : 76 kDa
    Observed band size : 85 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 100 kDa. We are unsure as to the identity of these extra bands.

References for Anti-GLB1 antibody (ab89084)

ab89084 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews or Questions for ab89084.
Please use the links above to contact us or submit feedback about this product.