HFE抗体

HFE抗体经ICC, IHC严格验证,品质保证.适用于多种种属反应性,被多篇文献引用并有用户反馈信息.确保特异性!产品具有以下特点:

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产品编号xy-12335R

英文名称HFE

中文名称遗传性血色病蛋白相关蛋白1抗体

别    名dJ221C16.10.1; Hemochromatosis; Hemochromatosis protein; Hereditary hemochromatosis protein; Hereditary hemochromatosis protein HLA H; HFE 1; HFE; HFE_HUMAN; HFE1; HH; High Fe; HLA H; HLA-H; HLAH; MGC:150812; MGC10379; MGC103790; MHC class I like protein HFE; MVCD7; TFQTL2.  

说 明 书100ul  200ul

研究领域肿瘤  心血管  细胞生物  神经生物学  信号转导  糖尿病  新陈代谢  

抗体来源Rabbit

克隆类型Polyclonal

HFE抗体交叉反应 Human, Mouse, Rat, Dog, Cow, Horse, Sheep,

产品应用ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 Flow-Cyt=1μg/Test 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.

分 子 量38kDa

细胞定位细胞膜

性    状Lyophilized or Liquid

浓    度1mg/1ml

免 疫 原KLH conjugated synthetic peptide derived from Human HFE/Hemochromatosis

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.

PubMedPubMed

产品介绍background:

The features of hemochromatosis include cirrhosis of the liver, diabetes, hypermelanotic pigmentation of the skin, and heart failure. Since hemochromatosis is a relatively easily treated disorder if diagnosed, this is a form of preventable cancer. The HFE protein, which is defective in hereditary hemo-chromatosis, normally is expressed in crypt enterocytes of the duodenum where it has a unique, predominantly intracellular localization. In placenta, the HFE protein co-localizes with and forms a stable association with the transferrin receptor (TfR), providing a link between the HFE protein and iron transport. Immunocytochemistry shows that the HFE protein and TfR both are expressed in the crypt enterocytes. Western blots show that, as is the case in human placenta, the HFE protein in crypt enterocytes is physically associated with the TfR and with β2-microglobulin. It is proposed that HFE has two mutually exclusive activities in cells: inhibition of uptake or inhibition of release of iron and that the balance between serum transferrin saturation and serum transferrin-receptor concentrations determines which of these functions predominates. The gene which encodes HFE maps to human chromosome 6p21.3.

Function:

Binds to transferrin receptor (TFR) and reduces its affinity for iron-loaded transferrin.

Subunit:

Binds TFR through the extracellular domain in a pH-dependent manner.

Subcellular Location:

Membrane; Single-pass type I membrane protein.

Tissue Specificity:

Expressed in all tissues tested except brain.

DISEASE:

Defects in HFE are a cause of hemochromatosis (HFE) [MIM:235200]. A disorder of iron metabolism characterized by iron overload. Excess iron is deposited in a variety of organs leading to their failure, and resulting in serious illnesses including cirrhosis, hepatomas, diabetes, cardiomyopathy, arthritis, and hypogonadotropic hypogonadism. Severe effects of the disease usually do not appear until after decades of progressive iron loading. Defects in HFE are associated with variegate porphyria (VP) [MIM:176200]. 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. VP is the most common form of porphyria in South Africa. It is characterized by skin hyperpigmentation and hypertrichosis, abdominal pain, tachycardia, hypertension and neuromuscular disturbances. High fecal levels of protoporphyrin and coproporphyrin, increased urine uroporphyrins and iron overload are typical markers of the disease. Note=Iron overload due to HFE mutations is a precipitating or exacerbating factor in variegate porphyria. Defects in HFE are associated with susceptibility to microvascular complications of diabetes type 7 (MVCD7) [MIM:612635]. These are pathological conditions that develop in numerous tissues and organs as a consequence of diabetes mellitus. They include diabetic retinopathy, diabetic nephropathy leading to end-stage renal disease, and diabetic neuropathy. Diabetic retinopathy remains the major cause of new-onset blindness among diabetic *****s. It is characterized by vascular permeability and increased tissue ischemia and angiogenesis.

Similarity:

Belongs to the MHC class I family.

Contains 1 Ig-like C1-type (immunoglobulin-like) domain.

Gene ID:

3077

抗体选择指南

HFE抗体检测任何目的靶蛋白都有不止一种抗体可供选择，为缩小抗体的选择范围选中合适的抗体，需要考虑如下几种因素：

（1） 分析或应用的类型

（2）样本蛋白的结构性质

（3）样本的种属

（4）抗体宿主的种类

（5）抗体的标记和检测

1 分析试验的应用类型一般抗体说明书都列出该抗体经试验验证过适用于何种分析类型，如：

可以应用于WB IHC ICC ELASA 分析等，如果抗体说明书没有提及的应用类型，并不意味着该抗体不适用于此种分析应用类型，而仅是说明尚未经过此种分析试验验证，如果抗体不适用某些分析试验，则会在抗体说明书上标注出来不适于某分析试验。

2 样本蛋白的结构性质了解样本蛋白的结构性质有助于选择*合适的抗体，至少需要考虑两方面因素

(1)..待测样本蛋白的结构域：HFE抗体是由各种不同**原**宿主而制备得来，其中的**原包括：全长蛋白、蛋白片断、多肽、全有机体（如：**）或细胞，抗体说明书一般都有**原的描述，如果打算检测的是蛋白片断或一种特殊的同型物或蛋白全长的某一区域，则必须选择用含此片段域的**原制备出的抗体。如果打算用FACS 流式检测活细胞的表面蛋白，则需要选择含该表面蛋白的胞外域来**制备的抗体。

(2)样本的提取或处理过程：某些抗体要求样本经过某些特殊处理，例如：许多抗体只识别还原和变性的、表位已暴露不受二级四级结构阻碍的蛋白样本，另一方面，某些抗体仅识别天然折叠状态的蛋白。

当选择**组化的抗体时，应注意某些抗体只识别未固定的冷冻的组织，而另一些抗体则适用于无需抗原修复解交联步聚的甲醛固定石蜡包埋的组织，这些都会在抗体说明书上应用部分标示出来 3 样本的物种 应选择物种相同或有交叉反应的抗体，抗体可能与不同物种的同种靶蛋白有交叉反应，因其氨基酸序列同源性较高。

如果样本的种类未列入抗体说明书上的交叉反应种属表中，并不意味着该抗体不适用于检测该物种的蛋白，而只是表示该物种尚未用此抗体检测验证过，应通过序列比对的方法来预测交叉反应，HFE抗体可应用Expasy 和 NCBI BLAST 来进行不同物种蛋白同源性比对。

4 一抗宿主物种的选择一般说来，在使用偶联二抗结合无偶联物的一抗时，一抗宿主动物的物种选择较为重要，对于**组化而言，尽可能选择与样本不同种系物种的一抗，从而避免二抗与样本内源性**球蛋白产生交叉反应，

例如：检测小鼠样本蛋白，则不应选择小鼠或大鼠源的一抗，*好选兔源的一抗，则二抗则可选择偶联了检测分子（酶、荧光素、生物素等）的抗兔IgG。如果选择有偶联物的一抗则不适用上述情况，除**组化外的其它对不含内源性**球蛋白样本的检测方法，则抗体宿主物种的影响不大，如对不含IgG 的细胞裂解物样本的western blotting检测。

尽管如此，HFE抗体含有血清的组织裂解物和组织培养上清中含有**球蛋白，还原变性样本中含IgG，在western blot 检测中则结合出现IgG 分子50 and 25 kDa 的重链和轻链条带。

5 二抗的选择 二抗应选用与使用的一抗相同的物种来源，例如：如果你的一抗是小鼠的单克隆抗体，二抗则选抗小鼠的二抗anti-mouse secondary。建议检查二抗说明书确保该抗体适用于你的检测应用， 二抗一般连接荧光素FITC 或发光团。

6 双重染色抗体的选择用未偶联一抗进行细胞培养物或组织切片的双重**染色要求一抗来源于不同物种并且二抗分别识别其中之一，二抗说明书应描述其与其它物种来源的**球蛋有否有交叉吸附。

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