铁螯合酶抗体
规格：1mg/1ml
英文名： FECH
别名： EPP; FCE; Ferrochelatase (protoporphyria); Ferrochelatase; Ferrochelatase mitochondrial; Heme synthetase; Protoheme ferro lyase; HEMH_HUMAN.
分子量： 43kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human EPB41
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,
细胞定位：细胞浆 细胞膜 线粒体
铁螯合酶抗体产品介绍：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.铁螯合酶抗体 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, 铁螯合酶抗体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. 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.
铁螯合酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域：心血管 细胞生物 **学 细胞类型标志物 细胞骨架 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid