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(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
人妻少妇啊灬啊灬用力啊快,高清黄色大片,FREE性欧美人与DOOG
Rabbit Anti-GCS1/Cy5.5 Conjugated antibody (bs-13322R-Cy5.5)
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(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-13322R-Cy5.5
英文名稱 Rabbit Anti-GCS1/Cy5.5 Conjugated antibody
中文名稱 Cy5.5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(shuō) 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(lái)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 www.ggggww.cn 北京博奧森生物技術(shù)有限公司
通過(guò)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(guò)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)
主站蜘蛛池模板: 中文字幕第一页卡通动漫在线 | 亚洲AV中文无码乱人伦在线R▽ | 性囗交免费视频观看 | 日本一级大毛片a一 | 天堂av一区二区 | 国产人妻一区二区三区四区五区六 | 少妇性BBB搡BBB爽爽爽视频 | 99精品免费视频 | 84pao国产成人高清在线 | 精品欧美一区二区在线观看欧美熟 | 国产精品入口 | 日韩精品卡1卡二卡3卡四卡 | 久久综合色视频 | 黄色在线视频网 | 欧美成人aaa级毛片在线视频 | 久久在线观看视频 | 欧美综合社区 | 国产精品三区www17con | 亚洲欧洲日产韩国在线看片 | zzijzzij亚洲日本少妇熟睡 | 日本三级影片 | 苍井空毛片精品久久久 | 久久久久久国产一级毛片... | 色丁香色婷婷 | 精精精精xxxx免费视频 | 国产精品俺来也在线观看 | 亚洲欧美成AⅤ人在线观看 aⅴ在天堂线网在线观看 | 国产资源视频在线观看 | 国产精品视频一区二区三区无码 | 国产天天草| 日本狠狠色 | 国产免费一区二区三区播放 | 海角在线观看91一区二区 | 中文字幕.av.在线 | 亚洲无人区在线观看AV | 精品国产乱码久久久久禁果 | 人妻忍着娇喘被中进中出视频 | 亚洲国产成人精品青青草原 | 无码少妇一区二区浪潮免费 | 麻豆传媒免费视频 | 国产亚洲亚洲高清视频 |