Overview

  • Product name

    Anti-GLB1/Beta-galactosidase antibody
    See all GLB1/Beta-galactosidase primary antibodies
  • Description

    Rabbit polyclonal to GLB1/Beta-galactosidase
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Cynomolgus monkey, Orangutan
  • Immunogen

    Recombinant fragment corresponding to Human GLB1/Beta-galactosidase aa 589-677.
    Sequence:

    GRYWPARGPQLTLFVPQHILMTSAPNTITVLELEWAPCSSDDPELCAVTF VDRPVIGSSVTYDHPSKPVEKRLMPPPPQKNKDSWLDHV


    Database link: P16278

  • Positive control

    • A431 cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab220283 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
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100.

Target

  • 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
  • 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

Images

  • Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized A431 cells labeling GLB1/Beta-galactosidase with ab220283 at 4 µg/mL (green).

References

ab220283 has not yet been referenced specifically in any publications.

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