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
一本久在线,黄色免费视频在线观看,午夜性刺激免费看视频
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-GCS1/Cy5 Conjugated antibody (bs-13322R-Cy5)
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-Cy5
英文名稱 Rabbit Anti-GCS1/Cy5 Conjugated antibody
中文名稱 Cy5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: 久久影院午夜 | 久久久九九 | av最新在线观看 | 浓精喷进老师黑色丝袜 | 人人揉人人捏人人爽五月天 | 少妇高潮太爽了在线观看欧美 | 天堂一区在线 | 被公牛日到了高潮 | 无码AV无码一区二区 | 好吊日好吊操在线视频 | 91久久夜色精品国产按摩 | 美女国产精品 | 不卡一不卡二不卡三 | 日韩人妻无码一区二区三区综合部 | 精品人妻无码区在线视频 | 希岛爱理av一区二区三区 | 国产91精品插插插免费观看 | 禁果av一区在线在观看 | a级片免费播放 | 禁果av一区在线在观看 | 扒开老师大腿猛进AAA片 | 日韩系列在线观看 | 亚洲大片精品 | 精品无码久久久久久午夜福利 | 国产成人亚洲精品无码Av大片 | 免费观看在线a级毛片 | 欧美精品欧美激情 | 亚洲夜色噜噜AV在线观看 | av鲁丝一区鲁丝二区鲁丝三区 | 欧美宗合 | 国产精品一品二区三区四区18 | 中文字幕精品无码综合网 | 大香伊蕉国产av | av免费人人干 | 女生毛片| 午夜不卡影院 | 簧片免费网站 | 自拍偷拍亚洲 | 99热国产在线手机精品 | 久久久久极品 | 中文在线好最新版在线 |