反义导向分子RGMC抗体
规格：1mg/1ml
英文名： RGMC/Repulsive Guidance Molecule C
别名： DL M; Haemojuvelin; HEMOCHROMATOSIS; HEMOCHROMATOSIS DUE TO DEFECT IN HEMOJUVELIN; HEMOCHROMATOSIS DUE TO DEFECT IN HEPCIDIN ANTIMICROBIAL PEPTIDE; HEMOCHROMATOSIS JUVENILE; Hemochromatosis type 2 (ju
分子量： 39kDa
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 Repulsiv
交叉反应：Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep,
细胞定位：细胞膜
反义导向分子RGMC抗体产品介绍：background: The repulsive guidance molecule (RGM) family of proteins are important in the guidance of growth cones of developing neurons. They are repulsive for a group of axons, those from the temporal half of the retina. RGM have been implicated in both axonal guidance and neural tube closure but as opposed to for ephrins, semaphorins, netrins and slits, no receptor mechanism for RGM activation has been defined. Dorsal root ganglion axons do not respond to RGM but neogenin (a netrin-binding protein which can function as an RGM receptor) expression can spur RGM responsiveness. The RGM proteins are attached to the membrane by a GPI-anchor. 反义导向分子RGMC抗体Two members of this family, RGMa and RGMb, are expressed in the nervous system. RGMc, also known as Hemojuvelin, is a part of the signaling pathway activating hepcidin and works together with hepcidin to restrict iron absorption in the gut. Defects in the gene encoding for RGMc causes the autosomal recessive disorder juvenile hemochromatosis (JH). Function: Mutations in Repulsive Guidance Molecule (also known as RGM-C; HJV; hemojuvelin; Hfe2) cause juvenile hemochromatosis, a severe iron overload disease. RGM-C gene expression has been characterized in the developing mouse and found to be exclusively expressed in all striated muscle and in the myocardium. Subunit: Interacts with BMP2 and BMP4. Interacts with BMPR1B. Interacts with TMPRSS6. Subcellular Location: Cell membrane; Lipid-anchor, GPI-anchor Tissue Specificity: Adult and反义导向分子RGMC抗体 fetal liver, heart, and skeletal muscle. DISEASE: Defects in HFE2 are the cause of hemochromatosis type 2A (HFE2A) [MIM:602390]; also known as juvenile hemochromatosis (JH). HFE2A is an early-onset autosomal recessive disorder due to severe iron overload resulting in hypogonadotrophic hypogonadism, hepatic fibrosis or cirrhosis and cardiomyopathy, occurring typically before age of 30. It is the consequence of intestinal iron hyperabsorption associated with macrophages that do not load iron. Deleterious mutations of HFE2 reduced HAMP (hepcidin) levels despite iron overload, which normally induces HAMP expression. Similarity: Belongs to the repulsive guidance molecule (RGM) family. Database links: UniProtKB/Swiss-Prot: Q6ZVN8.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
反义导向分子RGMC抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （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：细胞生物 发育生物学 神经生物学 信号转导 干细胞
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid