超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

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
国产精品色网,亚洲素人一区二区,日本视频又叫又爽
首頁 > 產品中心 > 一抗 > 產品信息
Beta galactosidase Rabbit pAb (bs-13369R)  
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
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價

產品編號 bs-13369R
英文名稱 Beta galactosidase Rabbit pAb
中文名稱 β-半乳糖苷酶1/β-Gal/彈性蛋白受體1抗體
別    名 BGAL_HUMAN; beta-galactosidase; GLB1; EC:3.2.1.23; Acid beta-galactosidase(Lactase); Elastin receptor 1; ELNR1; galactosidase beta 1; EBP; ELNR1; MPS4B; β-Galactosidase; β Galactosidase.  
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
克 隆 號
交叉反應 Rat (predicted: Human,Mouse,Pig,Sheep,Cow,Chicken,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 73 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Beta galactosidase: 301-400/677 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 This gene encodes a member of the glycosyl hydrolase 35 family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate the mature lysosomal enzyme. This enzyme catalyzes the hydrolysis of a terminal beta-linked galactose residue from ganglioside substrates and other glycoconjugates. Mutations in this gene may result in GM1-gangliosidosis and Morquio B syndrome. [provided by RefSeq, Nov 2015]

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.

SWISS:
P16278

Gene ID:
2720

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



產品圖片
Paraformaldehyde-fixed, paraffin embedded (rat testis tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (GLB1) Polyclonal Antibody, Unconjugated (bs-13369R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
版權所有 2004-2026 www.ggggww.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: 夜色资源ye321在线观看 | 国产高清学生妹在线观看视频一区 | 69精品久久久久9999不卡片 | 欧美一区二区三区诱惑在线 | 成人网在线播放 | 麻豆精品一二三区 | 国产亚洲一区二区视频 | jizz大全日本护士喷奶水 | 亚洲日韩成人 | 一区二区三区四区不卡在线 | 少妇嘿咻做爰吃奶摸视频网站 | 亚洲欧美日本国产综合 | 国产一区二区三区乱码在线观看 | 一区二区三区精品在线 | 久久精品成人免费视频 | 欧美日韩在线一区二区三区 | 91首页 | baoyu119.永久免费视频 | 国产在线不卡2005 | 国内精品久久久久久久久久清纯 | 少妇中文字幕乱码亚洲影视 | 日本极品 | 日本人妻丰满熟妇久久久久久 | 久久精品国产68国产精品亚洲 | 久久久精品午夜免费不卡 | 大又大粗又爽又黄少妇毛片免费 | 亚洲AV无码精品色午夜果冻不卡 | 国产成人精品久久久一区二区三区 | 国产在线拍偷自揄拍精品 | 激情视频91| 精品无人区无码乱码大片国产 | 陌陌影视在线观看免费 | 看国产黄大片在线观看 | 国产乱码精品一品二品 | 精品亚洲va在线va天堂资源站 | 永久免费av在线观看 | 久久久国产一级 | 人妻激情偷乱视频一区二区三区 | 中文字幕无码精品亚洲资源网久久 | 国产成人无码精品久久灭火器 | 久久久久久久久女人体 |