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  聯系我們
99re热视频在线,国产综合第一页,亚洲国产成人久久一区
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-FH/BF594 Conjugated antibody (bs-3938R-BF594)
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
產品編號 bs-3938R-BF594
英文名稱 Rabbit Anti-FH/BF594 Conjugated antibody
中文名稱 BF594標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  免疫學  轉錄調節因子  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 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 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.
版權所有 2004-2026 www.ggggww.cn 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         京公網安備110107000727號
主站蜘蛛池模板: 久久69国产精品久久69软件 | 日日猛噜噜狠狠扒开双腿小说 | av观看成片免费网站 | www.三区| 国产精品天天看天天狠 | 国产亚洲精品久久777777 | 啪啪网免费视频 | 日韩av免费 | 免费观看全黄做爰的视在线观看 | 一边吃奶一边做爰爽到爆视频 | 国产91影院 | 夜色资源站www国产在线观看 | 精产嫩模国品一二三区 | 熟女人妇交换俱乐部 | 婷婷开心深爱五月天播播 | 亚洲成人毛片 | 亚洲一区五码 | 成人影片亚区免费无码 | 在线免费观看一区二区三区 | 青青青视频香蕉在线观看视频 | 国产精品亚洲АV无码播放 闷骚的老熟女人15P | 国产精品一区二区三区四 | 无码精品国产一区二区三区免费 | 国产欧美精品一区二区三区介绍 | A级毛片高清免费视频在线播放 | av中文无码乱人伦 | 91精品婷婷国产综合久久 | 日本a不卡 | 久久青视频 | 全免一级毛片 | 一出一进一爽一粗一大视频免费的 | 嫩草影院网站在线 | 国产综合色在线 | 小12萝裸体视频国产 | 美国经典三级 | 久久精品国产68国产精品亚洲 | 国产精品久久国产三级国电话系列 | 日韩—二三区免费观看av | 色吧av色av | 国产成人亚洲综合a∨婷婷图片 | 中文精品一卡2卡3卡4卡国色 |