97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
国产免费观看视频,国产日产欧美一区二区,日日碰狠狠躁
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-GLB1/BF594 Conjugated antibody (bs-13369R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13369R-BF594
英文名稱 Rabbit Anti-GLB1/BF594 Conjugated antibody
中文名稱 BF594標記的β-半乳糖苷酶1/β-Gal/彈性蛋白受體1抗體
別    名 Acid beta galactosidase; Acid beta-galactosidase; Beta galactosidase 1; Beta galactosidase; Beta-galactosidase; BGAL_HUMAN; EBP; EBP, included; Elastin receptor 1 (67kD); Elastin receptor 1 67kDa; Elastin receptor 1; Elastin receptor 1, included; Elastin-binding protein, included; ELNR1; Galactosidase beta 1; GLB 1; GLB1; Lactase; MPS4B; S-GAL, included.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  轉運蛋白  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat,  (predicted: Human, Mouse, Chicken, Dog, Pig, Cow, Horse, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 73kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GLB1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
The human b-galactosidase gene, known as the LacZ gene, maps to chromosome 3p21.33 and encodes a 677 amino acid protein with an optimum functional pH range of 6 to 8. Catalytically active b-galactosidaseis (b-Gal) is a tetramer of four identical subunits, each with an active site, which can independently catalyze the cleavage of terminal galactose. Monovalent cations have a stimulatory effect on the enzymatic reaction, which likely involves a galactosyl-enzyme complex intermediate. b-Gals are widespread in animals, microorganisms and plants. The LacZ gene is widely used as a reporter gene with a variety of colored or fluorescent compounds capable of being produced from appropriate substrates, such as Xgal, which produces a blue color. For this reason, LacZ is incorporated into numerous plasmid vectors as a marker.

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.

Subcellular Location:
Lysosome and Cytoplasm > perinuclear region. Localized to the perinuclear area of the cytoplasm but not to lysosomes.

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.

Similarity:
Belongs to the glycosyl hydrolase 35 family.

Database links:

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

Unigene: 440489 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.kastlife.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 日韩人妻无码AV一区二区三区 | 日韩手机在线观看 | 青青网视频 | 色se02短视频永久网站 | 国产精品视频xxx | 成人国产三级 | 国产欧美亚洲精品 | 欧美性猛交xxxx免费看野外 | 涩爱av色老久久精品偷偷鲁 | 蜜桃国内精品久久久久软件9 | 在线高潮 | 欧美日韩精品一区二区三区高清视频 | 欧洲免费无线码在线一区 | 日韩欧美国产系列 | 国产精品第一页在线观看 | www.97超碰.com| 国产美女视频a级做爰色戎 一本久久A精品一区二区 | 男人被口爽还是啪啪爽 | 午夜剧场大片亚洲欧洲一区 | 亚洲成a人片777777久久 | 午夜男女爽爽影院网站 | 蜜桃国内精品久久久久软件9 | 青青视频免费观看 | 日本二三本的区别免费 | 亚洲国产成人精品女人久久久久 | 69久久夜色精品国产69 | 毛片18 | 人与动人物视频a级毛片 | 国产亚洲精品欧洲在线观看 | 日本69视频 | 久久国产午夜精品理论片推荐 | 在线观看av网 | 亚洲精一区二区三区 | 人人澡超碰碰97碰碰碰 | 裸体久久女人亚洲精品 | 国产亚洲欧美日韩在线一区 | 性色a∨人人爽网站 | 国产99久久久久久 | 国产精品国产三级国AV在线观看 | 国产精品对白一区二区三区 | 久久艹逼 |