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ABC 3; ABC C; ABC C transporter; ABC transporter 3; ABC-C transporter; ABC3; ABCA 3; Abca3; ABCA3 protein; ABCA3_HUMAN; ABCC; ATP binding cassette 3; ATP binding cassette sub family A (ABC1) member 3; ATP binding cassette sub family A member 3; ATP binding cassette transporter 3; ATP-binding cassette 3; ATP-binding cassette sub-family A member 3; ATP-binding cassette transporter 3; CED7. C. elegans, homolog of; EST111653; LBM 180; LBM180; MGC72201; SMDP3.  

研究领域肿瘤  细胞生物  信号转导  转运蛋白  结合蛋白  

抗体来源Rabbit

克隆类型Polyclonal

交叉反应(predicted: Human,Mouse,Rat,Dog,Cow,Sheep)

产品应用IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

理论分子量191kDa

细胞定位细胞膜 

性    状Liquid

浓    度1mg/ml

免 疫 原KLH conjugated synthetic peptide derived from human ABCA3/P180 Lamellar Body Protein: 65-160/1704 

亚    型IgG

纯化方法affinity purified by Protein A

缓 冲 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intracellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ABC1 subfamily. Members of the ABC1 subfamily comprise the only major ABC subfamily found exclusively in multicellular eukaryotes. The full transporter encoded by this gene may be involved in development of resistance to xenobiotics and engulfment during programmed cell death. [provided by RefSeq, Jul 2008]

Function:

Plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol.

Subcellular Location:

Membrane.

Tissue Specificity:

Highly expressed in lung, followed by brain, pancreas, skeletal muscle and heart. Weakly expressed in placenta, kidney and liver. Also expressed in medullary thyroid carcinoma cells (MTC) and in C-cell carcinoma.

DISEASE:

Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3) [MIM:610921]; also called pulmonary alveolar proteinosis due to ABCA3 deficiency. A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.

第一抗体就是平常所说的抗体,即能和抗原特异性结合。

第二抗体是能和抗体结合的,即抗体的抗体。主要用于检测抗体的存在。

一抗是针对抗原的抗体,二抗是针对一抗的抗体。即抗体也可以充当抗原刺激机体产生抗体。也就是说,抗原进入机体刺激机体产生免疫应答,由B细胞可以产生与相应抗原发生特异性结合的特殊蛋白质。

一抗二抗都是一种可以特异结合别的物质的基团,而且一抗可以至少结合两种其他基团(底物和二抗)。

一抗:可以特异结合底物,就是识别出我们想要检测的东西。一抗和底物结合与否用肉眼是看不出来的。

二抗:可以和一抗结合,并带有可以被检测出的标记(如带荧光、放射性、化学发光或显色基团),作用是检测一抗。 如果一抗自己带有可以被检测出的标记(如带荧光、放射性、化学发光或显色基团),则不需要二抗。但这样成本很高,因为一种一抗只识别一种底物。所以如今的设计一般是二抗带上可检测标记,再来检测一抗。而一抗识别底物。这样,当一抗结合到底物上,就可以通过二抗检测出来。

经x线晶体衍射结构分析发现，Ig由四条多肽链组成，各肽链之间由数量不等的链间二硫键连接。Ig可形成“Y”字型结构，称为Ig单体，是构成抗体的基本单位。

天然Ig分子含有四条异源性多肽链，其中，分子量较大的两条链称为重链(heavy chain，H)，而分子量较小的两条链称为轻链(Light chain，L)。同一Ig分子中的两条H链和两条L链的氨基酸组成相同。重链分子量为50KD～75KD，由450～550个氨基酸残基组成。由于重链恒定区的氨基酸组成和排列顺序不同，二硫键的数目和位置不同，其抗原性也不相同，可将Ig分为5类(class)，即IgM、IgD、IgG、IgA和IgE，其相应的重链分别为μ链、δ链、γ链、α链和ε链。轻链分子量约为25KD，由214个氨基酸残基构成。轻链可分为两种，分别为kappa(κ)链和lambda(λ)链。因此可将lg分为两型(type)，即κ型和λ型。正常人血清Ig的κ：λ约为2：1。

通过对不同Ig重链和轻链的氨基酸序列比较分析，发现其氨基端（N末端）氨基酸序列变化较大，称为可变区(variable region，V)，分别占重链和轻链的1/4和1/2；而羧基端（C末端）的氨基酸序列相对稳定的区域，称为恒定区(constant region，C)，分别占重链和轻链的3/4和1/2。

重链和轻链的V区分别称为VH和VL。VH和VL中各含有3个氨基酸组成和排列顺序高度可变的区域，称为高变区(HVR)或互补决定区(CDR)，包括HVR1(CDR1)、HVR2 (CDR2) 和HVR3 (CDR3)，其中HVR3 (CDR3)变化程度更高。VH的3个高变区分别位于29～31、49～58和95～102位氨基酸，而VL的3个高变区分别位于28～35、49～56和91～98位氨基酸。VH和VL的3个CDR共同组成Ig的抗原结合部位(antigen-binding site)，决定抗体的特异性，是抗体识别及结合抗原的部位。在V区中，CDR之外区域的氨基酸组成和排列顺序相对保守，称为骨架区(FR)。VH或VL各有四个骨架区，分别用 FR1、FR2、FR3和FR4表示。