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Rabbit Anti-ABCA3/P180 Lamellar Body Protein/BF594 Conjugated antibody (bs-17588R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號 bs-17588R-BF594
英文名稱 Rabbit Anti-ABCA3/P180 Lamellar Body Protein/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的三磷酸腺苷結(jié)合盒轉(zhuǎn)運(yùn)體3抗體
別    名 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.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
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 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 191kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ABCA3/P180 Lamellar Body Protein
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
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.

Similarity:
Belongs to the ABC transporter superfamily. ABCA family.
Contains 2 ABC transporter domains.

Database links:

Entrez Gene: 21 Human

Entrez Gene: 27410 Mouse

Entrez Gene: 302973 Rat

Omim: 601615 Human

SwissProt: Q99758 Human

SwissProt: Q8R420 Mouse

Unigene: 26630 Human

Unigene: 239470 Mouse

Unigene: 233424 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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