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货号： bs-6372R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-6372R-AF555
- 英文名称
- Anti-Hepcidin-25/AF555
- 中文名称
- AF555标记的铁调节蛋白25抗体
- 别 名
- Hamp; HEPC; HEPC_HUMAN; Hepc20; Hepc25; HEPCIDIN; Hepcidin; Hepcidin 20; Hepcidin 25; Hepcidin antimicrobial peptide; Hepcidin-20; Hepcidin25; HFE2; HFE2B; LEAP 1; LEAP-1; LEAP1; Liver expressed antimicrobial peptide; Liver-expressed antimicrobial peptide 1; PLTR; Putative liver tumor regressor.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 心血管 细胞生物 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 2/3kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Hepcidin-25
- 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 product encoded by this gene is involved in the maintenance of iron homeostasis, and it is necessary for the regulation of iron storage in macrophages, and for intestinal iron absorption. The preproprotein is post-translationally cleaved into mature peptides of 20, 22 and 25 amino acids, and these active peptides are rich in cysteines, which form intramolecular bonds that stabilize their beta-sheet structures. These peptides exhibit antimicrobial activity. Mutations in this gene cause hemochromatosis type 2B, also known as juvenile hemochromatosis, a disease caused by severe iron overload that results in cardiomyopathy, cirrhosis, and endocrine failure. [provided by RefSeq, Jul 2008].
Function:
Seems to act as a signaling molecule involved in the maintenance of iron homeostasis. Seems to be required in conjunction with HFE to regulate both intestinal iron absorption and iron storage in macrophages.Has strong antimicrobial activity against E.coli ML35P N.cinerea and weaker against S.epidermidis, S.aureus and group b streptococcus bacteria. Active against the fungus C.albicans. No activity against P.aeruginosa.
Involvement in disease;Defects in HAMP are the cause of hemochromatosis type 2B (HFE2B); also known as juvenile hemochromatosis (JH). HFE2B is a disorder of iron metabolism with excess deposition of iron in the tissues, bronze skin pigmentation, hepatic cirrhosis, arthropathy and diabetes. The most common symptoms of hemochromatosis type 2 at presentation are hypogonadism and cardiomyopathy.
Subcellular Location:
Secreted.
Tissue Specificity:
Highest expression in liver and to a lesser extent in heart and brain. Low levels in lung, tonsils, salivary gland, trachea, prostate gland, adrenal gland and thyroid gland. Secreted into the urine.
DISEASE:
Defects in HAMP are the cause of hemochromatosis type 2B (HFE2B) [MIM:613313]; also known as juvenile hemochromatosis (JH). HFE2B is a disorder of iron metabolism with excess deposition of iron in the tissues, bronze skin pigmentation, hepatic cirrhosis, arthropathy and diabetes. The most common symptoms of hemochromatosis type 2 at presentation are hypogonadism and cardiomyopathy.
Similarity:
Belongs to the hepcidin family.
Database links:Entrez Gene: 57817 Human
Entrez Gene: 84506 Mouse
Entrez Gene: 84604 Rat
Omim: 606464 Human
SwissProt: P81172 Human
SwissProt: Q9EQ21 Mouse
SwissProt: Q99MH3 Rat
Unigene: 8821 Human
Unigene: 7865 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Hepcidin是一种由肝脏细胞产生的具有广谱杀菌抑菌作用的抗菌肽，属于防御性蛋白家族。Hepcidin在肝脏合成时，首先产生一种早期多肽，由84个氨基酸残基组成。在其Gly24和Ser25之间存在一个酶切位点，当酶切去除其24个氨基酸残基的信号肽后，形成由60个氨基酸残基组成的前体肽(pro-Hepcidin)，并通过转运进入血液循环，最后由前肽转化酶(propeptide convertase)在N端的不同位点剪接生成3种不同的成熟小肽，分别由20、22、25个氨基酸组成，其中以25个氨基酸为其主要形式和主要活性部分，称作Hepcidin-25。含有25个氨基酸的小分子多肽蛋白主要在肝脏表达，其中有8个半胱氨酸---含25个氨基酸的小分子多肽蛋白是活性片段,和许多富含半胱的抗菌肽类似具有显著的抗菌作用。经研究表明Hepcidin25是机体铁稳态调控途径中关键性效应分子,其主要生物学功能在于调节小肠细胞对铁的吸收，也有学者称为：抗菌肽Hepcidin25。由于该蛋白多肽中有多个半胱氨酸构成，故导致二硫键的形成---形成多聚体蛋白，其分子量在20-32kDa。