强伦轩一级A片在线观看,中文字幕乱码人妻二区三区,鲁鲁狠狠狠7777一区二区,西西4444www无码精品
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  質量反饋  關于我們  聯(lián)系我們
精品国产人妻AV多野结衣,精品国产人妻AV多野结衣
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-Argininosuccinate Lyase/BF647 Conjugated antibody (bs-12515R-BF647)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-12515R-BF647
英文名稱 Rabbit Anti-Argininosuccinate Lyase/BF647 Conjugated antibody
中文名稱 BF647標記的琥珀酸裂解酶抗體
別    名 Argininosuccinase; Argininosuccinate lyase; Arginosuccinase; ARLY_HUMAN; ASAL; ASL; EC 4.3.2.1.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Horse, )
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 52kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ASL/Argininosuccinate Lyase
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 member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. A nontranscribed pseudogene is also located on the long arm of chromosome 22. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]

Function:
Amino-acid biosynthesis; L-arginine biosynthesis; L-arginine from L-ornithine and carbamoyl phosphate: step 3/3.
Nitrogen metabolism; urea cycle; L-arginine and fumarate from (N(omega)-L-arginino)succinate: step 1/1.

Subcellular Location:
Acetylation modifies enzyme activity in response to alterations of extracellular nutrient availability. Acetylation increased with trichostin A (TSA) or with nicotinamide (NAM). Glucose increases acetylation by about a factor of 3 with decreasing enzyme activity. Acetylation on Lys-288 is decreased on the addition of extra amino acids resulting in activation of enzyme activity.

DISEASE:
Defects in ASL are the cause of arginosuccinicaciduria (ARGINSA) [MIM:207900]. Arginosuccinicaciduria is an autosomal recessive disorder of the urea cycle. The disease is characterized by mental and physical retardation, liver enlargement, skin lesions, dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red, convulsions, and episodic unconsciousness.

Similarity:
Belongs to the lyase 1 family. Argininosuccinate lyase subfamily.

Database links:

Entrez Gene: 435 Human

Entrez Gene: 109900 Mouse

Entrez Gene: 59085 Rat

Entrez Gene: 512771 Cow

Omim: 608310 Human

SwissProt: Q3SZJ0 Cow

SwissProt: P04424 Human

SwissProt: Q91YI0 Mouse

SwissProt: P20673 Rat

Unigene: 632015 Human

Unigene: 23869 Mouse

Unigene: 64591 Rat



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.nmgps.com 北京博奧森生物技術有限公司
通過國際質量管理體系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
国产精品18禁久久久久久白浆 | 一区二区三区中文字幕 | 久久夜色精品国产欧美乱极品 | 欧产 日产 国产精品99 | 国产精品久久久爽爽爽麻豆色哟哟 | 国产传媒在线免费观看 | 久久人妻少妇嫩草av | 午夜成人免费视频网站 | 美女搡BBB又爽又猛又黄www | 极品嫩BBB槡BBBB | 国产高清无码视频在线观看 | 国产精品久久久久久久不卡 | 无码AV一区二区 | 国产AⅤ一区仑乱羞羞哒哒 91丨九色丨丰满熟女首页 | 国产人妻人伦精品无码.麻豆 | 亚洲AV无码成人精品区 | 国产精品成人AAAA网站女吊丝 | 成人国产AV无码一区二区 | AV岛国免费一区二区三区 | 东北女人无套内谢视频 | 黄色视频大全高清国产 | 字母数字黄片免费观看 | 亚洲乱熟乱熟女一区二区 | 四川BBB搡BBB爽爽爽欧美 | 在线观看亚洲大片短视频 | 女生粉嫩自慰网站 | 国产呻吟精品高潮久久AV无码 | 欧美经典成人视频在线看 | www.com黄色的网站污在线观看 | 蜜桃AV鲁一鲁一鲁一鲁樱花影院 | 成人精品一区二区三区中文字幕 | 91免费国产在线观看 | 国产黃色A片三区三区三小说 | 久久精品国产成人AV | 亚州成人av一区二区三区 | 免费的黄色视频网站下载在线 | 夏晴子无码一区二区三区 | 亚洲人成人无码网www国产 | 日韩A片一级无码免费 蜜桃 | 红桃视频乱码一区二区三区 | 国产日韩av高清无码 |