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: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human GLB1/Beta-galactosidase aa 315-365 conjugated to keyhole limpet haemocyanin. The exact sequence is proprietary.
    Sequence:

    AYWNGANSPYAAQPTSYDYDAPLSEAGDLTEKYFALRNIIQKFEKVPEGP I


    Database link: P16278

  • Positive control

    • Mouse embryo and liver tissue

Properties

Applications

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.

Application Abreviews Notes
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.

WB 1/100 - 1/500. Predicted molecular weight: 76 kDa.

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

  • 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.

References

This product has been referenced in:

  • 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). Read more (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). Read more (PubMed: 31239406) »
See all 4 Publications for this product

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