扫码关注公众号           扫码咨询技术支持           扫码咨询技术服务
  
客服热线:400-901-9800  客服QQ:4009019800  技术答疑  技术支持  质量反馈  关于我们  联系我们
产品中心-北京博奥森生物技术有限公司
首页 > 产品中心 > 一抗 > 产品信息
FECH Rabbit pAb (bs-9521R)  
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
技术支持:techsupport@bioss.com.cn
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-9521R
英文名称 FECH Rabbit pAb
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.  
Specific References  (1)     |     bs-9521R has been referenced in 1 publications.
[IF=2.589] Omoto K et al. Expression of peptide transporter 1 has a positive correlation in protoporphyrin IX accumulationinduced by 5-aminolevulinic acid with photodynamic detection of non-small cell lung cancer and metastatic brain tumor specimens originating from non-small cell lung cancer. Photodiagnosis Photodyn Ther. 2019 Mar;25:309-316.  IHC-P&WB ;  Human.  
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 (predicted: Human,Mouse,Rat,Pig,Sheep,Cow,Dog,Horse)
产品应用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:50-200,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 43 kDa
检测分子量
细胞定位 细胞浆 细胞膜 线粒体
性    状 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
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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



版权所有 2004-2026 www.bioss.com.cn 北京博奥森生物技术有限公司
通过国际质量管理体系ISO 9001:2015 GB/T 19001-2016    证书编号: 00124Q34771R2M/1100
通过国际医疗器械-质量管理体系ISO 13485:2016 GB/T 42061-2022    证书编号: CQC24QY10047R0M/1100
京ICP备05066980号-1         京公网安备110107000727号