Anti-GLB1/Beta-galactosidase抗体(ab96239)
Key features and details
- Rabbit polyclonal to GLB1/Beta-galactosidase
- Suitable for: WB, IHC-P
- Reacts with: Human
- Isotype: IgG
选择批间可重复性更高的重组抗体
- 研究可靠 —— 各批次间结果一致且可重复
- 长期批量供应 —— 采用重组技术,可实现快速生产
- 首次实验即可成功 —— 经过大量验证确认了特异性
- 符合伦理标准 —— 产品不含动物成分
概述
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产品名称
Anti-GLB1/Beta-galactosidase抗体
参阅全部 GLB1/Beta-galactosidase 一抗 -
描述
兔多克隆抗体to GLB1/Beta-galactosidase -
宿主
Rabbit -
经测试应用
适用于: WB, IHC-Pmore details -
种属反应性
与反应: Human -
免疫原
Recombinant fragment. This information is proprietary to Abcam and/or its suppliers.
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阳性对照
- H1299 cell lysates.
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常规说明
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
存储溶液
pH: 7.00
Preservative: 0.025% Proclin 300
Constituents: 78% PBS, 1% BSA, 20% Glycerol (glycerin, glycerine) -
Concentration information loading...
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纯度
Immunogen affinity purified -
克隆
多克隆 -
同种型
IgG -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab96239于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度;实际最佳的稀释度/浓度应由使用者检定。
应用 | Ab评论 | 说明 |
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WB |
1/500 - 1/3000. Predicted molecular weight: 76 kDa.
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IHC-P |
1/1000. Perform heat mediated antigen retrieval via the pressure cooker method before commencing with IHC staining protocol.
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说明 |
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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. |
靶标
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功能
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. -
疾病相关
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. -
序列相似性
Belongs to the glycosyl hydrolase 35 family. -
细胞定位
Lysosome and Cytoplasm > perinuclear region. Localized to the perinuclear area of the cytoplasm but not to lysosomes. - Information by UniProt
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数据库链接
- Entrez Gene: 2720 Human
- Omim: 611458 Human
- SwissProt: P16278 Human
- Unigene: 443031 Human
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别名
- Acid beta galactosidase antibody
- Acid beta-galactosidase antibody
- Beta galactosidase 1 antibody
see all
图片
数据表及文件
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SDS download
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Datasheet download
文献 (4)
ab96239 被引用在 4 文献中.
- Domingo-Vidal M et al. Cigarette Smoke Induces Metabolic Reprogramming of the Tumor Stroma in Head and Neck Squamous Cell Carcinoma. Mol Cancer Res 17:1893-1909 (2019). PubMed: 31239287
- Mills TA et al. Parathyroid Hormone-Related Peptide-Linked Hypercalcemia in a Melanoma Patient Treated With Ipilimumab: Hormone Source and Clinical and Metabolic Correlates. Semin Oncol 42:909-14 (2015). PubMed: 26615135
- Martinez-Outschoorn UE et al. Reverse Warburg effect in a patient with aggressive B-cell lymphoma: is lactic acidosis a paraneoplastic syndrome? Semin Oncol 40:403-18 (2013). PubMed: 23972703
- 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 . PubMed: 22684298