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(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
亚洲av首页在线,丁香五月天缴清在线,日日日b
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-GCS1/Cy3 Conjugated antibody (bs-13322R-Cy3)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)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-Cy3
英文名稱 Rabbit Anti-GCS1/Cy3 Conjugated antibody
中文名稱 Cy3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(lǐng)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(yīng) (predicted: Human, Mouse, Rat, Dog, )
產(chǎn)品應(yīng)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(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(quán)所有 2004-2026 www.ggggww.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)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(zhì)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: 一级a性色生活片久久毛片 国产精品香蕉 | 老师夹震蛋上课出白浆 | 日韩精品一区在线播放 | 飘雪影院手机免费观看免费动漫 | 在线超碰91| 成人性生交大片免费看视 | 男人av的天堂无码专区 | 狠狠色婷婷久久一区二区三区 | 日日噜噜噜噜夜夜爽 | 未成年人在线观看 | 久久中文字幕一区二区 | 免费观看全黄做爰的视在线观看 | 137大胆人体在线观看 | 国产成人精品在线观看 | 亚洲成A人片在线观看中文无码 | 日本老妇人乱xxy | 99国产精品久久久久久久夜 | 成人av网站免费观看 | 午夜影院免费 | 成年人视频在线免费 | 日日干夜 | 日韩精品一区二区在线天天狠天 | 欧美精品影院 | 亚洲天堂久久综合 | 美女网色 | 国产亚洲中字幕欧 | 日韩伦理中文字幕 | 欧洲精品成人免费视频在线观看 | 老熟女高潮一区二区三区 | 性荡视频播放在线视频7777 | 国产日韩综合性色av | 我的性奴的肉玩具1一17心奴 | 免费aaa级毛片在线视频 | 日韩乱码人妻无码系列中文字幕 | 国产在线国偷精品免费看 | 一区二区三区观看 | 韩国三级爽野战 | 国产成人精彩在线视频 | 国产福利一区二区在线观看 | 在线免费观看成人短视频 | 在线国产精品自拍 |