Anti-GLB1/Beta-galactosidase 抗体 [EPR8250] (ab128993)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR8250] to GLB1/Beta-galactosidase
- Suitable for: Flow Cyt (Intra), WB
- Reacts with: Human
Related conjugates and formulations
製品の概要
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製品名
Anti-GLB1/Beta-galactosidase antibody [EPR8250]
GLB1/Beta-galactosidase 一次抗体 製品一覧 -
製品の詳細
Rabbit monoclonal [EPR8250] to GLB1/Beta-galactosidase -
由来種
Rabbit -
アプリケーション
適用あり: Flow Cyt (Intra), WBmore details
適用なし: ICC/IF,IHC-P or IP -
種交差性
交差種: Human
交差が予測される動物種: Mouse, Rat -
免疫原
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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ポジティブ・コントロール
- SH-SY5Y, HeLa, Human placenta, HepG2 or BxPC-3 lysate, permeabilized HeLa cells.
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特記事項
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
製品の特性
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製品の状態
Liquid -
保存方法
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C. -
バッファー
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant -
Concentration information loading...
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精製度
Protein A purified -
ポリ/モノ
モノクローナル -
クローン名
EPR8250 -
アイソタイプ
IgG -
研究分野
関連製品
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Alternative Versions
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Isotype control
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Recombinant Protein
アプリケーション
The Abpromise guarantee
Abpromise保証は、 次のテスト済みアプリケーションにおけるab128993の使用に適用されます
アプリケーションノートには、推奨の開始希釈率がありますが、適切な希釈率につきましてはご検討ください。
アプリケーション | Abreviews | 特記事項 |
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Flow Cyt (Intra) |
1/10 - 1/100.
ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody. |
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WB |
1/10000 - 1/50000. Predicted molecular weight: 76 kDa.
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特記事項 |
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Flow Cyt (Intra)
1/10 - 1/100. ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody. |
WB
1/10000 - 1/50000. Predicted molecular weight: 76 kDa. |
ターゲット情報
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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
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参照データベース
- Entrez Gene: 2720 Human
- Entrez Gene: 12091 Mouse
- Entrez Gene: 316033 Rat
- Omim: 611458 Human
- SwissProt: P16278 Human
- SwissProt: P23780 Mouse
- Unigene: 443031 Human
- Unigene: 290516 Mouse
see all -
別名
- Acid beta galactosidase antibody
- Acid beta-galactosidase antibody
- Beta galactosidase 1 antibody
see all
画像
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All lanes : Anti-GLB1/Beta-galactosidase antibody [EPR8250] (ab128993) at 1/10000 dilution
Lane 1 : SH-SY5Y cell lysate
Lane 2 : HeLa cell lysate
Lane 3 : Human placenta cell lysate
Lane 4 : HepG2 cell lysate
Lane 5 : BxPC-3 cell lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : HRP labelled goat
anti-rabbit at 1/2000 dilution
Predicted band size: 76 kDa -
Intracellular flow cytometric analysis of permeabilized HeLa cells using ab128933 (red) or a rabbit IgG (negative) (green).
データシートおよび資料
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SDS download
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Datasheet download
参考文献 (4)
ab128993 は 4 報の論文で使用されています。
- Cui Y et al. Retromer has a selective function in cargo sorting via endosome transport carriers. J Cell Biol 218:615-631 (2019). PubMed: 30559172
- Mortensen ACL et al. The Stapled Peptide PM2 Stabilizes p53 Levels and Radiosensitizes Wild-Type p53 Cancer Cells. Front Oncol 9:923 (2019). PubMed: 31616635
- Yan Y et al. Laminin a4 overexpression in the anterior lens capsule may contribute to the senescence of human lens epithelial cells in age-related cataract. Aging (Albany NY) 11:2699-2723 (2019). PubMed: 31076560
- Xing J et al. Hypoxia induces senescence of bone marrow mesenchymal stem cells via altered gut microbiota. Nat Commun 9:2020 (2018). PubMed: 29789585