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
亚洲视频91,午夜看片在线观看,天天色天天搞
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-FH/BF488 Conjugated antibody (bs-3938R-BF488)
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-BF488
英文名稱 Rabbit Anti-FH/BF488 Conjugated antibody
中文名稱 BF488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: 国产亚洲天堂网 | 色偷偷成人网免费视频男人的天堂 | 亚洲一二区 | 一本色道久久HEZYO无码 | 精品国产乱码久久久久久影片 | 成人免费精品 | 欧美国产日本高清不卡 | 人妻无码αv中文字幕久久琪琪布 | 精品视频一区二区三区 | 国产一级片子 | 亚洲一区二区三区在线观看网站 | 免费在线激情视频 | 美国av毛片 | 国产成人久久精品77777的功能 | 国产精品久久久久久日本 | 天堂一区二区三区 | 黄色大片免费观看视频 | 成人性视频免费看的鲁片 | 国产精品一二三区在线观看 | 久久中文字幕av | 久久精品国产亚洲blacked | 少妇熟女高潮流白浆 | 日韩黄色av | 亚洲国产精品久久久 | 天天干天天曰天天操 | 国产丰满老熟女一区二区三区 | 国产午夜精品理论片久久影院 | 狠狠色丁香婷婷久久综合 | 亚洲自拍偷拍网站 | 日韩美女视频在线观看 | 亚洲天堂久久综合 | 夜夜躁狠狠躁日日躁麻豆护士 | 日韩精品久久理论片 | 亚洲性图 | 一本色道久久亚洲综合精品蜜桃 | 日韩在线一级片 | 他掀开裙子舌头伸进去在线观看 | 国产午夜啪啪 | 免费观看男女性高视频 | 欧美一级淫片a | 精品一区二区久久 |