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(lián)系我們
日本午夜高清,国产一级黄色录像片,欧美精美视频
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-GCS1/PE Conjugated antibody (bs-13322R-PE)
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(chǎn)品編號 bs-13322R-PE
英文名稱 Rabbit Anti-GCS1/PE Conjugated antibody
中文名稱 PE標記的β-葡萄糖苷酶1抗體
別    名 EC 3.2.1.106; glucosidase I; Mannosyl oligosaccharide glucosidase; Mannosyl-oligosaccharide glucosidase; Mogs; MOGS_HUMAN; Processing A glucosidase I; Processing A-glucosidase I.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  細胞類型標志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, )
產(chǎn)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 92kDa
細胞定位 細胞膜 
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GCS1
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(chǎn)品介紹 background:
Glycosylation of asparagine residues in Asn-X-Ser/Thr motifs in proteins commonly occur in the lumen of the endoplasmic reticulum (ER). Glucosidase I catalyzes the first step in the N-linked oligosaccharide processing pathway. It specifically removes the distal alpha 1,2-linked glucose residue from the Glc3-Man9-GlcNAc2 oligosaccharide precursor. Glucosidase I contains a short cytosolic tail, a single pass transmembrane domain and a large C-terminal catalytic domain located on the luminal side of the ER. Mutations in the gene encoding Glucosidase I result in the congenital disorder glycosylation (CDG-IIb), which is characterized by generalized hypotonia, dysmorphic features, hepatomegaly, hypoventilation, feeding problems, seizures and death. Two point mutations in the Glucosidase I gene have been identified and result in amino acid substitutions, namely Arg486Thr and Phe652Leu, that affect polypeptide folding and active site formation.

Function:
Cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner.

Subcellular Location:
Endoplasmic reticulum membrane.

DISEASE:
Defects in MOGS are the cause of type IIb congenital disorder of glycosylation (CDGIIb) [MIM:606056]; also known as glucosidase I deficiency. CDGIIb is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms of the infant included hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course was progressive and the infant did not survive more than a few months.

Similarity:
Belongs to the glycosyl hydrolase 63 family.

Database links:
UniProtKB/Swiss-Prot: Q13724.5

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.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(yī)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(wǎng)安備110107000727號
主站蜘蛛池模板: 豪放女大兵在线 | 99国产精品久久久久久久夜 | 亚洲porn | 日韩网站在线观看 | 日韩欧美中文字幕视频 | 狠狠综合久久久久尤物丿 | 亚洲AV无码不卡私人影院 | 免费精品自在久久 | yy6969久久亚洲精品 | 一级性感毛片 | 日韩乱码在线观看 | 奇米国产在线 | 在线观看亚洲AV日韩A∨ | 日本人在线看片免费观看 | 特级无码A级毛片特黄 | 欧美一区二区视频17c | 一级一级一片在线观看 | JAPANESE50MATURE亂倫 | 四虎影视久久久免费 | 久热综合在线亚洲精品 | 丰满岳乱妇在线观看中字无码 | 久久人操 | 国产黄色片在线免费观看 | 欧美xxxx三人交性视频 | 福利一区和二区 | 国产免费成人av | 人与牲口性恔配视频免费L 国产亚洲亚洲高清视频 | 看黄色a级片 | 欧美四级在线观看 | 久久精品aⅴ无码中文字字幕不卡 | 中文字幕人妻在线中文乱码怎么解决 | 久久69精品久久久久久国产越南 | 青青草原综合久久大伊人 | 色97色| 521色香蕉网站在线观看 | 国产一区在线视频播放 | 亚洲精品无码一区二区四区 | 黄带一级片| 国产成人鲁色资源国产91色综 | 日韩国产成人无码AV毛片 | 欧美日韩一卡2卡三卡4卡乱码欧美孕交 |