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货号： bs-11475R-PE-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-11475R-PE-Cy3
- 英文名称
- Anti-RGMC/Repulsive Guidance Molecule C/PE-Cy3
- 中文名称
- PE-Cy3标记的反义导向分子RGMC抗体
- 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 (juvenile); Hemochromatosis type 2; Hemochromatosis type 2 protein; Hemochromatosis type 2 protein homolog; HEMOCHROMATOSIS, TYPE 2A; HEMOCHROMATOSIS, TYPE 2B; Hemojuvelin; HFE 2; Hfe2; HFE2A; HJV; JH; Juvenile; MGC23953; Repulsive guidance molecule c; RGM C; RGM domain family member C; RGMC; RGMC_HUMAN.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 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
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Pig, Cow, Rabbit, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 39kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Repulsive Guidance Molecule C (205-255aa)
- 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.
- 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. 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 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.