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Product Name ABCA3/P180 Lamellar Body Protein Chinese Name 三磷酸腺苷结合盒转运体3抗体 Alias 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. literatures Specific References (1) | SL17588R has been referenced in 1 publications.[IF=3.098] Mingxing Fang. et al. SOX11 and FAK participate in the stretch‑induced mechanical injury to alveolar type 2 epithelial cells. Int J Mol Med. 2021 Jan;47(1):361-373 IF ; Mouse.Research Area Tumour Cell biology Signal transduction Transporter Binding protein Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Cow, Sheep, ) Applications IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 191kDa Cellular localization The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human ABCA3/P180 Lamellar Body Protein: 65-160/1704 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 1M TBS(pH7.4) with 1% BSA, 3% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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.
SWISS:
Q99758
Gene ID:
21
Database links:Entrez Gene: 21 Human
Entrez Gene: 27410 Mouse
Omim: 601615 Human
SwissProt: Q99758 Human
SwissProt: Q8R420 Mouse
Unigene: 26630 Human
Unigene: 239470 Mouse
Unigene: 233424 Rat
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