日日操日日操,亚洲一区二区三区四区视频,成人午夜福利激情,无码中文7幕

掃碼關(guān)注公眾號(hào)           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
丁香五月性爱激情网,加勒比一本道精品一区,日本久久久亚洲韩国精品一区
Rabbit Anti-GCS1/BF350 Conjugated antibody (bs-13322R-BF350)
訂購(gòu)熱線:400-901-9800
訂購(gòu)郵箱:sales@bioss.com.cn
訂購(gòu)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(jià)
產(chǎn)品編號(hào) bs-13322R-BF350
英文名稱 Rabbit Anti-GCS1/BF350 Conjugated antibody
中文名稱 BF350標(biāo)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(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  細(xì)胞類型標(biāo)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(chǔ) 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 bklrv.cn 北京博奧森生物技術(shù)有限公司
通過國(guó)際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號(hào): 00124Q34771R2M/1100
通過國(guó)際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
大鸡巴屌逼小视频| 国产一卡久久精品| 激情5月天91| 天天日天天干天天播| 欧美日韩一区二区不卡顿| 亚洲精品视频免费看| 无码av五月天| 久久久人妻96| 亚洲无码精品一区二区三区久噜噜噜| 粉嫩aⅴ一区二区三区四区五区| 高跟丝袜少妇被干图| 日本午夜第一区| 国内,欧美,日韩,成人| 超碰cao成人123| 国产成人久久久草草| 日本韩国一区二区| 国产精品视频中文字幕| 亚洲一区二区三区千人斩| 大香蕉8在线| 美女chab| 三级成人短视| 国产精品久久久久久三级苹果| 无码逼逼图片| 啪逼一区欧美日韩| 日本二区久久免费| 97碰碰久久720| 天天躁日日躁永久一区| 在线观看 图片区自拍偷拍| 女同区一区区二| 日韩精品 欧美 一区 二区| 中日韩在线久久久| 欧美成人黄片一区二区| 一区二区三区四区不卡在线视频| 无码久久免费电影| 黄色小说视频在线观看网站| 亚洲高清精品一区二区| 大香蕉aⅴ| 天天干91porn| 国模欧美另类在线| 麻精品秘 国产| 后入巨乳人妻少妇|