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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.  

研究領(lǐng)域腫瘤  細(xì)胞生物  信號轉(zhuǎn)導(dǎo)  轉(zhuǎn)運(yùn)蛋白  結(jié)合蛋白  

抗體來源Rabbit

克隆類型Polyclonal

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

產(chǎn)品應(yīng)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.

理論分子量191kDa

細(xì)胞定位細(xì)胞膜 

性    狀Liquid

濃    度1mg/ml

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

亞    型IgG

純化方法affinity purified by Protein A

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

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

注意事項(xiàng)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.

第一抗體就是平常所說的抗體,即能和抗原特異性結(jié)合。

第二抗體是能和抗體結(jié)合的,即抗體的抗體。主要用于檢測抗體的存在。

一抗是針對抗原的抗體,二抗是針對一抗的抗體。即抗體也可以充當(dāng)抗原刺激機(jī)體產(chǎn)生抗體。也就是說,抗原進(jìn)入機(jī)體刺激機(jī)體產(chǎn)生免疫應(yīng)答,由B細(xì)胞可以產(chǎn)生與相應(yīng)抗原發(fā)生特異性結(jié)合的特殊蛋白質(zhì)。

一抗二抗都是一種可以特異結(jié)合別的物質(zhì)的基團(tuán),而且一抗可以至少結(jié)合兩種其他基團(tuán)(底物和二抗)。

一抗:可以特異結(jié)合底物,就是識別出我們想要檢測的東西。一抗和底物結(jié)合與否用肉眼是看不出來的。

二抗:可以和一抗結(jié)合,并帶有可以被檢測出的標(biāo)記(如帶熒光、放射性、化學(xué)發(fā)光或顯色基團(tuán)),作用是檢測一抗。 如果一抗自己帶有可以被檢測出的標(biāo)記(如帶熒光、放射性、化學(xué)發(fā)光或顯色基團(tuán)),則不需要二抗。但這樣成本很高,因?yàn)橐环N一抗只識別一種底物。所以如今的設(shè)計(jì)一般是二抗帶上可檢測標(biāo)記,再來檢測一抗。而一抗識別底物。這樣,當(dāng)一抗結(jié)合到底物上,就可以通過二抗檢測出來。

經(jīng)x線晶體衍射結(jié)構(gòu)分析發(fā)現(xiàn)，Ig由四條多肽鏈組成，各肽鏈之間由數(shù)量不等的鏈間二硫鍵連接。Ig可形成“Y"字型結(jié)構(gòu)，稱為Ig單體，是構(gòu)成抗體的基本單位。

天然Ig分子含有四條異源性多肽鏈，其中，分子量較大的兩條鏈稱為重鏈(heavy chain，H)，而分子量較小的兩條鏈稱為輕鏈(Light chain，L)。同一Ig分子中的兩條H鏈和兩條L鏈的氨基酸組成相同。重鏈分子量為50KD～75KD，由450～550個(gè)氨基酸殘基組成。由于重鏈恒定區(qū)的氨基酸組成和排列順序不同，二硫鍵的數(shù)目和位置不同，其抗原性也不相同，可將Ig分為5類(class)，即IgM、IgD、IgG、IgA和IgE，其相應(yīng)的重鏈分別為μ鏈、δ鏈、γ鏈、α鏈和ε鏈。輕鏈分子量約為25KD，由214個(gè)氨基酸殘基構(gòu)成。輕鏈可分為兩種，分別為kappa(κ)鏈和lambda(λ)鏈。因此可將lg分為兩型(type)，即κ型和λ型。正常人血清Ig的κ：λ約為2：1。

通過對不同Ig重鏈和輕鏈的氨基酸序列比較分析，發(fā)現(xiàn)其氨基端（N末端）氨基酸序列變化較大，稱為可變區(qū)(variable region，V)，分別占重鏈和輕鏈的1/4和1/2；而羧基端（C末端）的氨基酸序列相對穩(wěn)定的區(qū)域，稱為恒定區(qū)(constant region，C)，分別占重鏈和輕鏈的3/4和1/2。

重鏈和輕鏈的V區(qū)分別稱為VH和VL。VH和VL中各含有3個(gè)氨基酸組成和排列順序高度可變的區(qū)域，稱為高變區(qū)(HVR)或互補(bǔ)決定區(qū)(CDR)，包括HVR1(CDR1)、HVR2 (CDR2) 和HVR3 (CDR3)，其中HVR3 (CDR3)變化程度更高。VH的3個(gè)高變區(qū)分別位于29～31、49～58和95～102位氨基酸，而VL的3個(gè)高變區(qū)分別位于28～35、49～56和91～98位氨基酸。VH和VL的3個(gè)CDR共同組成Ig的抗原結(jié)合部位(antigen-binding site)，決定抗體的特異性，是抗體識別及結(jié)合抗原的部位。在V區(qū)中，CDR之外區(qū)域的氨基酸組成和排列順序相對保守，稱為骨架區(qū)(FR)。VH或VL各有四個(gè)骨架區(qū)，分別用 FR1、FR2、FR3和FR4表示。