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  聯系我們
操操图片视频第一网,最新亚洲AV成人网站在线观看,日本成人在线免费
首頁 > 產品中心 > 一抗 > 產品信息
Coproporphyrinogen III Oxidase Rabbit pAb (bs-8860R)  
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
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價
產品編號 bs-8860R
英文名稱 Coproporphyrinogen III Oxidase Rabbit pAb
中文名稱 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.  
研究領域 細胞生物  發育生物學  干細胞  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Chicken,Dog)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 39 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CPOX/Coproporphyrinogen Oxidase: 361-454/454 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
P36551

Gene ID:
1371

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



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: 国产欧美日韩精品一区 | 日韩精品一区二区三 | 女人下边被添全过程A片 | 91精品久久久 | 久久字幕精品一区 | 久久99久久99精品免视看看 | 久久久久久成人毛片免费看 | 青青草华人在线 | 欧美成人影片 | 日本少妇人妻XXXXⅩ18 | 欧美另类激情 | 欧美成人A片无码免费 | 上锁的房间中国版免费 | www.国产91 | 亚洲欧美日韩一区二区在线观看 | 精品国产高清一区二区三区 | 国产亚洲精品AA片在线不卡 | 国产精品男人影院在线播放 | 成人一区在线观看 | 国产精品人妻熟女a8198v久 | 野花在线观看免费 | 日女av在线 | 亚洲精品无码久久久久久久 | 国产自精品 | 青草福利 | 国产一区国产二区在线观看 | 国产在线精品国自产拍影院同性 | 一二三区不卡 | 国产盗摄XXXX视频XXXⅩ | 亚洲AV成人午夜一区二区 | 午夜久久福利 | 少妇大叫又粗又大太爽A片 heyzo朝桐光一区二区 | 激情另类小说区图片区视频区 | 国产日韩欧美一区二区三区乱码 | 一区二区三区日本 | 天堂网www天堂在线资源库 | 成人av色| 提莫影院AV毛片入口 | 男人操女人高清视频 | 在线观看亚洲欧美视频免费 | 国产色产综合色产在线观看视频 |