吃奶呻吟打开双腿做受动态图 -亚洲色偷偷色噜噜狠狠99网-日韩精品极品视频在线观看免费-来一水AV@lysav

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(zhì)量反饋  人才招聘  關于我們  聯(lián)系我們
永久免费精品精品永久-夜色,亂倫近親相姦中文字幕 ,日韩A片无码毛片免费看小说
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-SLC19A3/PE-Cy5 Conjugated antibody (bs-8702R-PE-Cy5)
訂購熱線:400-901-9800
訂購郵箱:sales@xucheq.com
訂購QQ:  400-901-9800
技術支持:techsupport@xucheq.com
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-8702R-PE-Cy5
英文名稱1 Rabbit Anti-SLC19A3/PE-Cy5 Conjugated antibody
中文名稱 PE-Cy5標記的溶質(zhì)載體家族19成員3抗體
別    名 Solute carrier family 19 member 3; Thiamine transporter 2; thTr 2; THTR2.  
規(guī)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  神經(jīng)生物學  信號轉導  轉運蛋白  跨膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,  (predicted: Human, Rat, )
產(chǎn)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 56kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SLC19A3
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:
This gene encodes a ubiquitously expressed transmembrane thiamine transporter that lacks folate transport activity. Mutations in this gene cause biotin-responsive basal ganglia disease (BBGD); a recessive disorder manifested in childhood that progresses to chronic encephalopathy, dystonia, quadriparesis, and death if untreated. Patients with BBGD have bilateral necrosis in the head of the caudate nucleus and in the putamen. Administration of high doses of biotin in the early progression of the disorder eliminates pathological symptoms while delayed treatment results in residual paraparesis, mild mental retardation, or dystonia. Administration of thiamine is ineffective in the treatment of this disorder. Experiments have failed to show that this protein can transport biotin. Mutations in this gene also cause a Wernicke's-like encephalopathy.[provided by RefSeq, Jan 2010]

Function:
Mediates high affinity thiamine uptake, propably via a proton anti-port mechanism. Has no folate transport activity.

Subcellular Location:
Plasma membrane.

Tissue Specificity:
Widely expressed but most abundant in placenta, kidney and liver.

DISEASE:
The disease is caused by mutations affecting the gene represented in this entry.
Disease description:An autosomal recessive metabolic disorder characterized by episodic encephalopathy, often triggered by febrile illness, presenting as confusion, seizures, external ophthalmoplegia, dysphagia, and sometimes coma and death. If untreated, encephalopathies can result in permanent dystonia. Brain imaging may show characteristic bilateral lesions of the basal ganglia.

Similarity:
Belongs to the reduced folate carrier (RFC) transporter (TC 2.A.48) family.

Database links:

Entrez Gene: 80704 Human

Omim: 606152 Human

SwissProt: Q9BZV2 Human

Unigene: 221597 Human



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.xucheq.com 北京博奧森生物技術有限公司
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
我强行与岳的性关系| 高清欧美性猛交XXXX黑人猛交| 少妇人妻AV| 欧洲熟妇色XXXXX欧美老妇伦| 日本三级欧美三级人妇视频黑白配| 国产精品Ⅴ无码大片在线看| 国产精品一区二区AV| 久久人人爽爽人人爽人人片AV| 韩国三级日本三级人与波| 色综合久久网| 国产毛片久久久久久国产毛片| 不用播放器的AV| 国产精品无码电影在线观看| 4444亚洲人成无码网在线观看| 荫蒂每天被男人添| 在线精品国产一区二区三区 | 狠狠色综合7777久夜色撩人| 熟妇人妻无乱码中文字幕| BL漫画网站| 国产成人一区二区三区影 | 精品久久久久久无码免费| 欧美性色黄大片A级毛片视频| 日本理伦片午夜理伦片| FREEXX性黑人大战欧美视频| 国产精品人人爽人人做我的可爱| 国语精彩对白在线视频| 久久精品国产亚洲AV麻豆蜜芽| XX性欧美肥妇精品久久久久久| 免费a级黄毛片| 久久久久无码专区亚洲AV| 国产av人人夜夜澡人人爽麻豆| 99久久精品免费看国产| 免费无遮挡无码永久在线观看视频| 女人和拘做受全过程免费| 久久精品国产亚洲AV忘忧草18| 亚洲国产成人片在线观看无码| 色哟哟网站入口在线观看视频| 国产伦精品一区二区三区| 宝宝好涨水快流出来免费视频| 欧美性受XXXX白人性爽| 婷婷丁香五月|