Anti-FECH抗体上海钰博生物科技有限公司

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产品名称: Anti-FECH抗体
英文名称: FECH
抗体货号: YB--9521R
产品规格: 20ul/50ul/100ul/200ul
级    别: 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 1580元
抗原: KLH conjugated synthetic peptide derived from human EPB41:81-180/423
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/5/21 15:33:00
详细资料:  实验方法技术资料
浏览人数:66
诚信指数:1658点
了解更多:进入公司展台
使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Anti-FECH抗体

品编号 YB-9521R
英文名称 FECH
中文名称 铁螯合酶抗体
别    名 EPP; FCE; Ferrochelatase (protoporphyria); Ferrochelatase; Ferrochelatase mitochondrial; Heme synthetase; Protoheme ferro lyase; HEMH_HUMAN.  
规格价格 100ul/1580元 购买    200ul/2480元 购买    大包装/询价
说 明 书 100ul  200ul
研究领域 心血管  细胞生物  免疫学  细胞类型标志物  细胞骨架  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:50-200 (石蜡切片需做抗原修复) 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 43kDa
细胞定位 细胞浆 细胞膜 线粒体
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human EPB41:81-180/423 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
PubMed PubMed
产品介绍 background:
Ferrochelatase catalyzes the insertion of the ferrous form of iron into protoporphyrin IX in the heme synthesis pathway, and is localised in the mitochondrion. Defects in ferrochelatase are associated with protoporphyria. Two transcript variants encoding different isoforms have been found for this gene. Porphyrias are a group of inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). They are broadly classified as hepatic porphyrias or erythropoietic porphyrias, based on the site of the overproduction and mainly accumulation of the porphyrins (or their chemical precursors). They manifest with either skin problems, or neurological complications, or occasionally both.

Function:
Catalyzes the ferrous insertion into protoporphyrin IX. 

Subunit:
Homodimer. 

Subcellular Location:
Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

DISEASE:
Defects in FECH are the cause of erythropoietic protoporphyria (EPP) [MIM:177000]. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. EPP is a form of porphyria marked by excessive protoporphyrin in erythrocytes, plasma, liver and feces, and by widely varying photosensitive skin changes ranging from a burning or pruritic sensation to erythema, edema and wheals. 

Similarity:
Belongs to the ferrochelatase family. 

SWISS:
P22830

Gene ID:
2235

Database links:

Entrez Gene: 2235 Human

Omim: 612386 Human

SwissProt: P22830 Human

Unigene: 365365 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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