97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

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
在线观看av不卡,国产一区二区三区午夜,午夜黄色免费网站
首頁 > 產(chǎn)品中心 > 標記一抗 > 產(chǎn)品信息
Rabbit Anti-Coproporphyrinogen III Oxidase/Biotin Conjugated antibody (bs-8860R-Bio)
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-8860R-Bio
英文名稱 Rabbit Anti-Coproporphyrinogen III Oxidase/Biotin Conjugated antibody
中文名稱 生物素標記的原卟啉氧化酶3抗體
別    名 Coprogen oxidase; COPROPORPHYRIA; Coproporphyrinogen III oxidase; Coproporphyrinogenase; COX; CPO; CPOX; CPX; HEM 6; Hem-6; Hem6; HEM6_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  發(fā)育生物學  干細胞  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, Sheep, )
產(chǎn)品應用 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 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 39kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CPOX/Coproporphyrinogen Oxidase
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:
CPOX is a 454 amino acid mitochondrial enzyme that is localized to the inner membrane space of erythrocytes. It participates in the sixth step of heme biosynthesis by catalyzing the formation of protoporphyrinogen IX from copropophyrinogen III. Mutations in the gene encoding CPOX are the cause of coproporphyria, an autosomal dominant disease characterized by skin photosensitivity and neurological disturbances. Symptoms are often experienced as attacks, which include severe abdominal and nerve pain. People affected by coproporphyria overexcrete copropophyrinogen III in feces and urine and the enzymatic activity of CPOX is found to be approximately half that of normal, leading to a decrease in overall heme synthesis. There is no cure for coproporphyria, but preventative treatment to relieve symptoms usually involves dietary changes and avoidance of drugs and alcohol.

Function:
Coproporphyrinogen Oxidase (CPOX) is the sixth enzyme of the heme biosynthetic pathway. This soluble protein is localized in the intermembrane space of mitochondria and its catalytic activy mediates the coversion of Coproporphyrinogen-III + O2 + 2 H+ into Protoporphyrinogen-IX + 2 CO2 + 2 H2O. Defects in CPOX are the cause of hereditary coproporphyria

Subunit:
Homodimer.

Subcellular Location:
Mitochondrion intermembrane space.

DISEASE:
Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase activity.

Similarity:
Belongs to the aerobic coproporphyrinogen-III oxidase family.

Database links:

Entrez Gene: 1371 Human

Entrez Gene: 12892 Mouse

Entrez Gene: 304024 Rat

Omim: 612732 Human

SwissProt: P36551 Human

SwissProt: P36552 Mouse

SwissProt: Q3B7D0 Rat

Unigene: 476982 Human

Unigene: 291519 Mouse

Unigene: 19581 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.kastlife.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(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
主站蜘蛛池模板: 黄色天堂在线观看 | 亚洲小说综合网 | av九九 | 欧美视频亚洲视频 | 国产高清成人久久 | 无码人妻一区二区三区免费N鬼沢 | 久久不见久久见www免费视频 | 一本久道久久综合狠狠爱 | 奶头和荫蒂添的好舒服囗交 | 国产精品69久久久久孕妇欧美 | 国内精品久久久人妻中文字幕 | av免费在线播放网址 | 一级全黄男女免费大片 | 国产精品久久久久无码人妻 | 日本少妇做爰全过程二区 | 麻豆视频观看免费视频观看 | 91麻豆免费版 | 国产人妻精品区一区二区 | 野花在线观看免费 | 可以免费看的av毛片 | 麻豆传媒视频在线播放 | 国产www网站 | 日本啪啪网站永久免费 | 一区二区免费在线观看 | 亚洲美女视频 | 女人本色免费观看 | 无码AV午夜福利一区 | 精品无码国产AV一区二区 | 国产又粗又硬又猛的毛片视频 | 两个人看的www免费 国产一区二区三区18 | 桃子视频在线观看免费视频网 | 五月丁香六月激情综合在线视频 | 国产女合集小岁9三部 | 亚洲乱码中文字幕小综合 | 日韩高清www| 红杏出墙在线 | 国产h在线观看 | jizz成熟丰满韩国女人 | 国产一二三区在线视频 | 中日韩亚洲人成无码网站 | av一区在线|