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
一区影视,成人在线视频一区二区,91综合视频在线观看
Rabbit Anti-FH/Biotin Conjugated antibody (bs-3938R-Bio)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購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-3938R-Bio
英文名稱 Rabbit Anti-FH/Biotin Conjugated antibody
中文名稱 生物素標(biāo)記的富馬酸水合酶抗體
別    名 mitochondrial; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=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 51kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Fumarate hydratase
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:
The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. [provided by RefSeq, Jul 2008]

Function:
Also acts as a tumor suppressor.

Subunit:
Homotetramer.

Subcellular Location:
Cytoplasm, Mitochondrion

Tissue Specificity:
Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.

DISEASE:
Fumarase deficiency (FMRD): The disease is caused by mutations affecting the gene represented in this entry. A severe autosomal recessive metabolic disorder characterized by early-onset hypotonia, profound psychomotor retardation, and brain abnormalities, such as agenesis of the corpus callosum, gyral defects, and ventriculomegaly. Many patients show neonatal distress, metabolic acidosis, and/or encephalopathy.
Hereditary leiomyomatosis and renal cell cancer (HLRCC): The disease is caused by mutations affecting the gene represented in this entry. A disorder characterized by predisposition to cutaneous and uterine leiomyomas, and papillary type 2 renal cancer which occurs in about 20% of patients.

Similarity:
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.

Database links:

Entrez Gene: 520260 Cow

Entrez Gene: 480092 Dog

Entrez Gene: 2271 Human

Entrez Gene: 14194 Mouse

Entrez Gene: 100627128 Pig

Entrez Gene: 24368 Rat

Entrez Gene: 393938 Zebrafish

Omim: 136850 Human

SwissProt: P07954 Human

SwissProt: P97807 Mouse

SwissProt: P10173 Pig

SwissProt: P14408 Rat

SwissProt: Q7SX99 Zebrafish

Unigene: 592490 Human

Unigene: 41502 Mouse

Unigene: 29782 Rat

Unigene: 104452 Zebrafish



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
通過國際質(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: 人人妻人人玩人人澡人人爽 | 日韩欧美精品人妻一区二区不卡 | 国产情趣视频在线观看 | 国产精品片aa在线观看 | 麻豆专媒体一区二区 | 国产亚洲精品久久久久的角色 | 铠甲勇士第一部52集全 | 福利精品在线观看 | 午夜一区欧美二区高清三区 | mmm·www看片免费观看 | 男女做爰猛烈视频大尺度小说 | 1000精品久久久久久久久 | 超鹏97国语| 国产农村乱 | 日韩欧美一区二区三区在线 | 欧美亚州综合 | 欧美日韩亚洲视频 | 山东中年夫妇大白天露脸自拍 | 玖玖玖在线观看 | 综合AV第1页 | 欧美26uuu免费视频 | 欧洲精品成人免费视频在线观看 | 国产69精品久久久久乱码韩国 | 色一情一乱一伦 | 国产福利姬喷水福利在线观看 | 亚洲色最新高清AV网站 | 成人在线视频一区二区 | 欧美1级片网站 | 在线视频国产一区 | 美女免费黄视频 | 成人三级黄色片 | 亚洲春色Aⅴ无码专区在线播放 | 少妇人妻上班偷人精品视频 | 亚洲交一区 | 国产在线高清视频无码不卡互動交流 | 女人与动zzz0000xxxx | 国产成人A∨麻豆精品 | 色中色登陆器 | 自拍偷拍亚洲欧美 | 68精品国产免费久久久久久婷婷 | 斗破苍穹年番在线观看 |