亚洲制服欧美另类-午夜激情av电影-日本高清中文字幕一区二区三区-中国欧美日韩一区二区三区-欧洲亚洲日本韩国-成人欧美激情一区二区-亚洲偷偷自拍高清

掃碼關(guān)注公眾號(hào)           掃碼咨詢(xún)技術(shù)支持           掃碼咨詢(xún)技術(shù)服務(wù)
  
客服熱線(xiàn):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
日韩一区二无码专区播放,日本不卡高字幕在线2019
Rabbit Anti-OTC/Ornithine Carbamoyltransferase/PE-Cy5 Conjugated antibody (bs-18037R-PE-Cy5)
訂購(gòu)熱線(xiàn):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(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-18037R-PE-Cy5
英文名稱(chēng) Rabbit Anti-OTC/Ornithine Carbamoyltransferase/PE-Cy5 Conjugated antibody
中文名稱(chēng) PE-Cy5標(biāo)記的鳥(niǎo)氨酸氨甲?;€(xiàn)粒體抗體
別    名 EC 2.1.3.3; MGC129967; MGC129968; MGC138856; OCTD; Ornithine carbamoyltransferase mitochondrial; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  新陳代謝  線(xiàn)粒體  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) Mouse, Rat,  (predicted: Human, Dog, Pig, Cow, Horse, Sheep, )
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 36kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human OTC/Ornithine Carbamoyltransferase
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:
This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008]

Subcellular Location:
Mitochondrion matrix.

Tissue Specificity:
Mainly expressed in liver and intestinal mucosa.

Post-translational modifications:
Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.

DISEASE:
Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.

Similarity:
Belongs to the ATCase/OTCase family.

Database links:

Entrez Gene: 280887 Cow

Entrez Gene: 480878 Dog

Entrez Gene: 5009 Human

Entrez Gene: 18416 Mouse

Entrez Gene: 397438 Pig

Entrez Gene: 25611 Rat

Entrez Gene: 101102207 Sheep

Omim: 300461 Human

SwissProt: Q9N1U7 Cow

SwissProt: P00480 Human

SwissProt: P11725 Mouse

SwissProt: O19072 Pig

SwissProt: P00481 Rat

SwissProt: P84010 Sheep

Unigene: 117050 Human

Unigene: 2611 Mouse

Unigene: 2391 Rat



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.a6308.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(shū)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(shū)編號(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
中国女人精69xxxxxx视频 | 少妇荡乳情欲办公室456视频 | 动漫AV网站免费观看 | 99精品无人区乱码1区2区3区 | 久久国产免费观看精品1 | 国产91精品系列在线观看 | 7788精品视频免费观看 | 亚洲二区在线视频 | 四虎国产精品永久地址入口 | 麻婆豆传媒一区二区三 | 国模无码一区二区三区 | 18视频入口网站在线看 | 国产美女久久久久久久久久久 | 久久久久久久国产免费看 | 麻豆文化传媒精品1区2区3区 | 99国产精品一区二区三区四区五区 | 国内精品一区二区综合欧美 | 国内精品一区二区综合欧美 | 欧美成人精品欧美一级乱黄 | 国产爱豆果冻传媒在线观看 | 久久久久久久久久国产精品免费 | 亚洲国际精品素人AV | 国产在线精彩视频 | 老汉扛起娇妻玉腿进入 | 厨房掀起裙子从后面进去视频 | 91麻豆精品国产一级 | 国产日韩欧美丝袜一区二区 | 偷窥欧美wc经典tv | 国产精品午夜免费观看网站 | 鲁人人人鲁人人鲁精品 | 爆乳3把你榨干哦OVA在线观看 | 精品日产卡一卡二卡麻豆 | 少妇无码太爽了不卡视频在线看 | 毛片成人永久免费视频 | XXX2高清在线观看免费视频 | 一本色道久久综合亚洲精品加 | 国产a视频精品免费观看 | 四川小少妇BBAABBAA | 美女性爽视频国产免费 | 亚洲1区2区3区精华液 | 亚洲AV日韩AV无码AV一区二区三区 |