在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

Human β-galactosidase,βGAL ELISA Kit

  • 中文名稱:
    人β半乳糖苷酶(βGAL)酶聯免疫試劑盒
  • 貨號:
    CSB-E09463h
  • 規格:
    96T/48T
  • 價格:
    ¥3600/¥2500
  • 其他:

產品詳情

  • 產品描述:
    人β半乳糖苷酶(βGAL)酶聯免疫試劑盒(CSB-E09463h)為雙抗夾心法ELISA試劑盒,定量檢測血清、血漿、尿液、組織勻漿樣本中的GLB1含量。GLB1基因編碼β-半乳糖苷酶,與多種疾病相關。研究發現,其突變會導致β-半乳糖苷酶功能缺陷,引發GM1神經節苷脂貯積癥和黏多糖貯積癥IVB型等疾病。對其研究機制主要聚焦在解析突變影響及探索酶替代治療等方面。試劑盒檢測范圍為7.8 μlU/mL-500 μlU/mL,適用于基礎科研中探索βGAL在細胞代謝調控、衰老標記物分析或疾病模型中的表達水平變化;通過尿液分析探究代謝異常機制,或利用組織勻漿研究特定病理條件下溶酶體功能狀態;為生物醫學領域研究βGAL相關分子機制及生物標志物篩選提供有效工具本品僅用于科研,不用于臨床診斷,產品具體參數及操作步驟詳見產品說明書。
  • 別名:
    Acid beta galactosidase ELISA Kit; Acid beta-galactosidase ELISA Kit; Beta galactosidase 1 ELISA Kit; Beta galactosidase ELISA Kit; Beta-galactosidase ELISA Kit; BGAL_HUMAN ELISA Kit; EBP ELISA Kit; EBP; included ELISA Kit; Elastin receptor 1 (67kD) ELISA Kit; Elastin receptor 1 67kDa ELISA Kit; Elastin receptor 1 ELISA Kit; Elastin receptor 1; included ELISA Kit; Elastin-binding protein; included ELISA Kit; ELNR1 ELISA Kit; Galactosidase beta 1 ELISA Kit; GLB 1 ELISA Kit; GLB1 ELISA Kit; Lactase ELISA Kit; MPS4B ELISA Kit; S-GAL; included ELISA Kit
  • 縮寫:
    GLB1
  • Uniprot No.:
  • 種屬:
    Homo sapiens (Human)
  • 樣本類型:
    serum, plasma, urine, tissue homogenates
  • 檢測范圍:
    7.8 μlU/mL-500 μlU/mL
  • 靈敏度:
    1.95 μlU/mL
  • 反應時間:
    1-5h
  • 樣本體積:
    50-100ul
  • 檢測波長:
    450 nm
  • 研究領域:
    Immunology
  • 測定原理:
    quantitative
  • 測定方法:
    Sandwich
  • 精密度:
    Intra-assay Precision (Precision within an assay): CV%<8%      
    Three samples of known concentration were tested twenty times on one plate to assess.  
    Inter-assay Precision (Precision between assays): CV%<10%      
    Three samples of known concentration were tested in twenty assays to assess.    
                 
  • 線性度:
    To assess the linearity of the assay, samples were spiked with high concentrations of human βGAL in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
      Sample Serum(n=4)  
    1:5 Average % 100  
    Range % 95-110  
    1:10 Average % 97  
    Range % 92-101  
    1:20 Average % 86  
    Range % 82-90  
    1:40 Average % 93  
    Range % 86-98  
  • 回收率:
    The recovery of human βGAL spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
    Sample Type Average % Recovery Range  
    Serum (n=5) 95 89-98  
    EDTA plasma (n=4) 92 88-96  
                 
                 
  • 標準曲線:
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
    µlU/ml OD1 OD2 Average Corrected  
    500 2.046 1.952 1.999 1.800  
    250 1.742 1.665 1.704 1.505  
    125 1.365 1.404 1.385 1.186  
    62.5 1.057 1.109 1.083 0.884  
    31.2 0.824 0.786 0.805 0.606  
    15.6 0.511 0.538 0.525 0.326  
    7.8 0.342 0.329 0.336 0.137  
    0 0.201 0.197 0.199    
  • 數據處理:
  • 貨期:
    3-5 working days

產品評價

靶點詳情

  • 功能:
    Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans.; 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.
  • 基因功能參考文獻:
    1. We confirmed a diagnosis of GM1 gangliosidosis based on GLB1 mutations and/or the deficiency of beta-galactosidase activity. We identified the first two cases by whole-exome sequencing, and then the other six cases by direct sequencing of GLB1 with enzyme analysis. The recurrent mutation, p.D448V in GLB1, accounted for 50.0% of total alleles in our cohort. PMID: 29439846
    2. GLB1 rs4678680 SNP contributes to susceptibility to develop HBV-related hepatocellular carcinoma PMID: 27489354
    3. beta-Gal expression in articular cartilage is associated with progressive knee osteoarthritis joint damage and is a potential indictor of disease severity. PMID: 26112901
    4. This study shows that moderate widespread expression of betagal in the CNS of GM1 gangliosidosis mice is sufficient to achieve significant biochemical impact with phenotypic amelioration and extension in lifespan PMID: 25964428
    5. The study proposes an explanation for ELNR1 uncoupling based on the age-related alterations of Neu-1 activity. PMID: 26086247
    6. Overexpression of the novel senescence marker GLB1 in prostate cancer predicts reduced recurrence of PSA-expressing tumors. PMID: 25876105
    7. Identification and analysis of GLB1 mutations in Indian patients with GM1 gangliosidosis. PMID: 25936995
    8. We observed significant lower values of beta-galactosidase, FUC and tendency to decrease of MAN and GLU concentration in nasal polyps PMID: 23911047
    9. This study analyzed patient cells with GM1 gangliosidosis and sialidosis. A novel mutation p.E186A is identified in GLB1 gene. PMID: 25600812
    10. The activity of serum GAL was significantly higher in colon cancer patients with a history of alcohol and nicotine dependence. PMID: 24018455
    11. beta-galactosidase, considered as a senescence marker, is over-expressed only in specific subtypes of pituitary adenomas, but is also present in carcinomas considered as a group PMID: 22908062
    12. In a Turkish population, mutations in GLB1 gene leads to severely deficient enzyme activity and result in infantile phenotype of the GM1 gangliosidosis. PMID: 22234367
    13. GLB alleles have a role in GM1-gangliosidosis and Morquio B disease, and fluorous iminoalditols act as effective pharmacological chaperones against their gene products PMID: 22033734
    14. Elastin derived peptides may play a role in neovascular age-related macular degeneration by binding to and inducing neovascular phenotypes in choroidal endothelial cells through their receptor, GLB1. PMID: 22178079
    15. The non-random distribution of plasma membrane-associated beta-hexosaminidase and beta-galactosidase and their localization within lipid microdomains, suggest a role of these enzymes in the local reorganization of glycosphingolipid-based signalling units. PMID: 21978926
    16. We show here that mouse GLB1 has greater stability when compared to human GLB1, and that human GLB1 activity is temperature and protective-dependent on protein cathepsin A, while that of mouse GLB1 is not PMID: 22001501
    17. Crystal structure of human beta-galactosidase: structural basis of Gm1 gangliosidosis and morquio B diseases. PMID: 22128166
    18. Plasma beta-galactosidase and beta-hexosaminidase levels are higher in patients with Alzheimer's disease-type 2 diabetes mellitus (T2DM) compared to those with T2DM alone. PMID: 21321400
    19. luciferase-based assay is a reliable and convenient method for screening and evaluation of chaperone effects on human beta-gal mutants PMID: 20826101
    20. Results describe four mutations in Han Chinese patients that induce significant suppression of beta-galactosidase activity, correlating with severity of GM1 gangliosidosis and presence of cardiomyopathy. PMID: 20920281
    21. Data show that four mutations of GLB1 could be correlated to a distinct GM1 gangliosidosis phenotype. PMID: 20175788
    22. The hyperthermia-enhanced association between tropoelastin and its 67-kDa chaperone results in better deposition of elastic fibers. PMID: 20947500
    23. Three new mutations in three Morquio B patients where the Trp 273 Leu mutation is absent PMID: 12393180
    24. This protein was expressed locally in the media and adventitia at injected arterial segments without any significant dissemination to remote areas. PMID: 12515396
    25. polymorphisms in beta 1 galactosidase is associted with type-II GM1 gangliosidosis PMID: 12644936
    26. the 67-kDa elastin receptor was specifically expressed in the epithelioid or multinucleated giant cells in giant cell granuloma PMID: 14987258
    27. 4 new GLB1 mutations were found: a premature stop codon in exon 2 (c.171C>G); a splicing error in intron 2 (c.245+1G>A); missense mutation in exon 4 (c.451G>T); & a splicing mutation in intron 8 (c.914+4A>G). PMID: 15365997
    28. 4 new and 10 known GLB1 mutations were studied. The c.1445G>A (p.Arg482His), c.175C>T (p.Arg59Cys), c.733+2T>C, c.1736G>A (p.Gly579Asp), & c.1051C>T (p.Arg351X) mutations, affect the stabilization of PPCA by hampering the interaction of GLB1/EBP & PPCA. PMID: 15714521
    29. expression and number of El-R on white blood cells using a specific 67 kDa El-R antibody, and presence of mRNA corresponding to the gene coding for El-R PMID: 15907791
    30. Infantile impaired elastogenesis arose from a primary elatin binding protein (ELNR1) defectin gangliosidosis, according to molecular analysis. PMID: 16314480
    31. effects of GLB1, PPCA and NEU1 gene mutations on elastogenesis in skin fibroblasts PMID: 16538002
    32. Senescence Associated-beta-gal activity is expressed from GLB1, the gene encoding lysosomal beta-D-galactosidase, the activity of which is typically measured at acidic pH 4.5. PMID: 16626397
    33. Twenty-one novel mutations in the GLB1 gene identified in a large group of GM1-gangliosidosis and Morquio B patients suggesting common origin for the prevalent p.R59H mutation among gypsies. PMID: 16941474
    34. 14 novel mutations in the GLB1 gene were identified in patients with GM1 gangliosidosis from Argentina PMID: 17309651
    35. Senescence-associated beta-galactosidase activity in human melanocytic nevi is absent in vivo. PMID: 17522702
    36. Here we report the new variant p.Arg595Trp in the GLB1 gene, which markedly reduces beta-galactosidase activity when expressed in COS-1 cells. The variant was identified in the healthy father of a girl with GM1 gangliosidosis PMID: 17661814
    37. GLB1 mutant alleles have roles in GM1-gangliosidosis and Morquio B patients PMID: 17664528
    38. canine model is an appropriate animal model for the human late infantile form and represents a versatile system to test gene therapeutic approaches for human and canine G(M1)-gangliosidosis PMID: 18088383
    39. Mutation responsible for feline G(M1) gangliosidosis was identified in beta-galactosidase resulting in an amino acid substitution at arginine 483, known to cause G(M1) gangliosidosis in humans. PMID: 18353697
    40. 102 mutations distributed along the beta-galactosidase gene have been reported in GM1 gangliosidosis patients. PMID: 18524657
    41. Report deregulation of versican and elastin binding protein in solar elastosis. PMID: 18704747
    42. Neuraminidase caused the desialylation of both PDGF and IGF-1 receptors and diminished the intracellular signals induced by the mitogenic ligands PDGF-BB and IGF-2. PMID: 18772331
    43. Elastin receptor-mediated monocyte chemoattraction induced by polysaccharide from Candida and seaweed. PMID: 18976701
    44. GLB1 mutation caused Morquio type B disease with mental regression in two siblings. PMID: 19091613
    45. missense mutations affecting the catalytic site of acid beta-galactosidase in Morquio B disease PMID: 19472408
    46. in skin, the activation of the S-Gal/Cath-A/Neu-1 "elastin receptor" complex might dictate cell survival and skin tissue repair PMID: 19769716

    顯示更多

    收起更多

  • 相關疾病:
    GM1-gangliosidosis 1 (GM1G1); GM1-gangliosidosis 2 (GM1G2); GM1-gangliosidosis 3 (GM1G3); Mucopolysaccharidosis 4B (MPS4B)
  • 亞細胞定位:
    [Isoform 1]: Lysosome.; [Isoform 2]: Cytoplasm, perinuclear region.
  • 蛋白家族:
    Glycosyl hydrolase 35 family
  • 組織特異性:
    Detected in placenta (at protein level). Detected in fibroblasts and testis.
  • 數據庫鏈接:

    HGNC: 4298

    OMIM: 230500

    KEGG: hsa:2720

    STRING: 9606.ENSP00000306920

    UniGene: Hs.443031



主站蜘蛛池模板: 亚洲色中文字幕在线播放| 欧美激情第1页| 99视频精品全部免费 在线| 国产成人午夜福利在线小电影| 亚洲爆乳成av人在线视水卜| 久久久久人妻一区精品色| 日韩成av人片在线观看| 老色鬼永久视频网站| 亚洲精品国产一二三无码av| 超碰aⅴ人人做人人爽欧美| av天堂永久资源网| 中文字幕乱码一区二区三区免费 | 中文字幕有码无码人妻在线| 亚洲精品久久久久久久蜜桃臀| 国产黄大片在线观看画质优化| 久久香蕉国产线看观看怡红院妓院| 人妻精品动漫h无码网站| 午夜福利精品导航凹凸| 色爱无码av综合区| 丰满少妇被粗大的猛烈进出视频| 国产免费福利在线视频| 亚洲高清毛片一区二区| а√资源新版在线天堂| 毛片完整版的免费观看| 无码一区二区免费波多野播放搜索 | 国产精品岛国久久久久| 国产成人av激情在线播放| 伊人久久大香线蕉av五月天| 国产成人无码一区二区在线观看| 成人网站免费大全日韩国产| 国产超碰女人任你爽| 久久精品一区二区免费播放| 欧美精品久久久久久久自慰| 免费人成视频网站在线观看18| 麻豆成人久久精品综合网址| 亚洲乱亚洲乱妇50p| 国产亚洲精品久久久久久打不开| 亚洲大码熟女在线观看| 国产成人a∨激情视频厨房| 日韩人妻无码一区二区三区99| 亚洲色在线无码国产精品|