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(xún)技術(shù)支持           掃碼咨詢(xún)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
亚洲AV大乳天堂在线观看,风韵丰满熟妇啪啪区老老熟妇,日本专区
Rabbit Anti-FH/BF647 Conjugated antibody (bs-3938R-BF647)
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(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-3938R-BF647
英文名稱(chēng) Rabbit Anti-FH/BF647 Conjugated antibody
中文名稱(chēng) BF647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(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 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(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 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(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)
主站蜘蛛池模板: 国产成人精品久久亚洲高清不卡p | 久久艹色 | 国产69精品久久久久99 | 成人精品视频一区二区三区尤物 | 亚洲精品深夜AV无码一区二区 | 国产欧美日韩一区2区 | jiujiure精品视频播放 | 久久久精品在线 | 日韩精品卡1卡二卡3卡四卡 | 福利姬国产在线观看免费 | 欧美喷潮久久久XXXXX | 一级高清 | 亚洲欧美日韩综合久久久久久 | 粉嫩av一区二区三区四区五区 | 久久精品夜夜夜夜夜久久 | 精品熟女少妇av免费久久 | 国产视频一区二区三区四区 | 久久夜色精品国产www | 2018天天干天天操 | 久久国产免费 | 国产JJZZJJZZ视频全部免费 | 免费无码专区毛片高潮喷水 | 亚洲成人福利网 | zzijzzij亚洲日本少妇熟睡 | 无码一卡二卡三卡四卡 | 一国产一级淫片a免费播放口 | 粉嫩av一区二区三区四区五区 | 色就是色成人网 | 亚洲午夜精品一区二区三区四区 | 色999久久久精品人人澡69 | 999精品欧美一区二区三区黑人 | 欧美日韩特级黄片观看 | 国产一精品一aⅴ一免费 | 欧美色图国产精品 | 国产精品9191 | 中文字幕爆乳JULIA女教师 | 精品国产福利久久久 | av在线中文播放 | 日韩精品视频成人 | 天堂一区二区三区 | 麻豆影视免费观看 |