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: 1区2区3区欧美日本 午夜男女羞羞爽爽爽视频 婷婷五月六月激情综合色中文字幕 | www.欧美成| 国产亚洲av片在线观看18女人 | 国产午夜视频免费 | 欧美人牲口杂交在线播放免费 | 中文国产日韩欧美二视频 | 久草在线免费看视频 | 国产精品高潮在线 | 区一区二在线观看 | 91av网址| 免费观看黄色网页 | ai杨幂被弄高潮在线观看 | 久久精品视频导航 | 成人无码A片一区二区三区免费看 | 偷窥目拍性综合图区 | 久久精品国产精品亚洲色婷婷 | 无码国产精品一区二区免费虚拟VR | 9191久久 | 亚洲熟妇无码一区二区三区导航 | xxxx中国一级片 | 色就是色成人网 | 中文在线a | 成人做爽爽爽爽免费国产软件 | 69xx免费视频 | 成人亚洲 | 无码少妇精品一区二区免费动态 | 国产欧美日韩精品第一区二区三区 | 亚洲成人一区在线观看 | 亚洲国产成人乱码 | WWW亚洲色大成网络.COM | 殴美性猛交| 边啃奶头边躁狠狠躁玩爽在水里面 | 国产毛片毛多水多的特级毛片 | yp最新网站入口 | 日产精品一区二区 | 图片区小说区另类春色 | 国产成人精彩在线视频 | 99久久久精品免费观看国产 | 在线这里只有精品 | 农村少妇好紧水多视频 | 国产亚洲综合AA系列 |