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
7777精品伊人久久久大香线蕉,欧美成性色,黄色一级片性生活
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-GCS1/PE-Cy5.5 Conjugated antibody (bs-13322R-PE-Cy5.5)
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-13322R-PE-Cy5.5
英文名稱 Rabbit Anti-GCS1/PE-Cy5.5 Conjugated antibody
中文名稱 PE-Cy5.5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
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: 日韩精品人妻系列无码专区免费 | 国模无码大尺度一区二区三区 | 亚洲精品欧美视频 | 麻豆爱爱| 视频二区一区 | ai杨幂被弄高潮在线观看 | 国产999精品久久久久久绿帽 | 午夜精品久久久久久久男人的天堂 | 第一福利所导航 | 992tv在线观看免费进 | 日本国产视频 | 天下免费大全正版资料 | 日韩视频精品在线 | 亚色av| 国产日产欧洲无码视频无遮挡 | 一区二区三区影视 | 一区二区三区 | 日本一本久草 | 亚洲a色 | 亚洲色成人一区二区三区小说 | 少妇毛片久久久久久久久 | 精品在线视频一区二区三区 | 无码伊人久久大杳蕉中文无码 | 在线亚洲观看 | 在线播放免费人成视频在线观看 | 国产综合激情 | 粉嫩av一区二区老牛影视 | 中文字幕人成一区 | 欧美性猛交xxxx乱大交俱乐部 | 国产99在线观看 | 第84页国产精品 | 日韩精品亚洲专区在线观看 | 边摸边吃奶边做爽gif动态视频 | 免费网站成人亚洲 | AV不卡在线永久免费观看 | 欧美亚洲一区在线观看 | 亚洲五月天综合 | 亚洲AV无码一区二区三区18 | 伊人中文字幕无码专区 | 亚洲国产精品原创巨作AV | 天堂一区二区三区 |