ABCA3/P180 Lamellar Body Protein,三磷酸腺苷结合盒转运体3抗体

产品编号HZ-17588R
英文名称ABCA3/P180 Lamellar Body Protein
中文名称三磷酸腺苷结合盒转运体3抗体
别 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.
说 明 书0.1ml 0.2ml
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Human, Mouse, Rat, Dog, Cow, Sheep,
ABCA3/P180 Lamellar Body Protein,三磷酸腺苷结合盒转运体3抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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191kDa
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 ABCA3/P180 Lamellar Body Protein
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.
ABCA3/P180 Lamellar Body Protein,三磷酸腺苷结合盒转运体3抗体PubMedPubMed
产品介绍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.

Subunit:

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.

ABCA3/P180 Lamellar Body Protein,三磷酸腺苷结合盒转运体3抗体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.

Gene ID:
21

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.