製品の概要

  • 製品名Anti-GLB1 antibody
    GLB1 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to GLB1
  • アプリケーション適用あり: WB, IHC-Pmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Recombinant protein fragment corresponding to a region within amino acids 166 and 652 of Human GLB1.

  • ポジティブ・コントロール
    • H1299 cell lysates.

法規制情報

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab96239 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
WB 1/500 - 1/3000. Predicted molecular weight: 76 kDa.
IHC-P 1/1000. Perform heat mediated antigen retrieval via the pressure cooker method before commencing with IHC staining protocol. PMID: 22684298

ターゲット情報

  • 機能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.
  • 関連疾患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.
  • 配列類似性Belongs to the glycosyl hydrolase 35 family.
  • 細胞内局在Lysosome and Cytoplasm > perinuclear region. Localized to the perinuclear area of the cytoplasm but not to lysosomes.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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 画像

  • Anti-GLB1 antibody (ab96239) at 1/1000 dilution + H1299 whole cell lysate at 30 µg

    Predicted band size : 76 kDa

Anti-GLB1 antibody (ab96239) 使用論文

This product has been referenced in:
  • Capparelli C  et al. Autophagy and senescence in cancer-associated fibroblasts metabolically supports tumor growth and metastasis via glycolysis and ketone production. Cell Cycle 11:2285-302 (2012). IHC-P ; Human . Read more (PubMed: 22684298) »

See 1 Publication for this product

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Thank you for your reply.

Yes, we would guarantee this antibody in IHC-P since it has been published to work. I hope this information helps. Please contact us with any other questions.

Thank you for contacting us.

We do not test this antibody in IHC-P in house, only in WB. I've forwarded the publication to our references team to review. This is the first we've heard of ab96239 being used in IHC-P, but we may add this to ou...

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