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CFTR Rabbit pAb (bs-1277R)  
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產(chǎn)品編號 bs-1277R
英文名稱 CFTR Rabbit pAb
中文名稱 囊性纖維化跨膜轉(zhuǎn)運調(diào)節(jié)因子抗體
別    名 ABC 35; ABC35; ABCC 7; ABCC7; ATP binding cassette sub family C member 7; ATP Binding Cassette Superfamily C Member 7; ATP binding cassette transporter sub family C member 7; cAMP dependent chloride channel; CF; CFTR/MRP; Channel conductance controlling ATPase; Cystic fibrosis transmembrane conductance regulator; Cystic fibrosis transmembrane conductance regulator ATP binding cassette sub family C member 7; ATP-binding cassette sub-family C member 7; cAMP-dependent chloride channel; CFTR; CFTR_HUMAN; Channel conductance-controlling ATPase; Cystic Fibrosis Transmembrane Regulator; Cystic Fibrosis Transmembrane Regulator; dJ760C5.1; MRP 7; MRP7; TNR CFTR.  
Specific References  (4)     |     bs-1277R has been referenced in 4 publications.
[IF=4.141] Junfeng He. et al. Distribution and Expression of Pulmonary Ionocyte-Related Factors CFTR, ATP6V0D2, and ATP6V1C2 in the Lungs of Yaks at Different Ages. GENES-BASEL. 2023 Mar;14(3):597  IHC ;  Bovine.  
[IF=3.553] Liu M et al. Copper promotes sheep pancreatic duct organoid growth by ATOX1-dependent MEK-ERK pathway activation. Am J Physiol Cell Physiol. 2020 Apr 1;318(4):C806-C816.  IF ;  sheep.  
[IF=3.553] Liu M et al. Copper promotes sheep pancreatic duct organoid growth by ATOX1-dependent MEK-ERK pathway activation. Am J Physiol Cell Physiol. 2020 Apr 1;318(4):C806-C816.  IF ;  sheep.  
[IF=3.23] Ren, Hui, Nigel P. Birch, and Vinod Suresh. "An Optimised Human Cell Culture Model for Alveolar Epithelial Transport." PloS one 11.10 (2016): e0165225.  WB ;  Human.  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,Rat (predicted: Mouse,Rabbit,Pig,Sheep,Cow,Chicken,Dog,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 168 kDa
檢測分子量
細(xì)胞定位 細(xì)胞漿 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CFTR: 145-280/1480 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 This gene encodes a member of the ATP-binding cassette (ABC) transporter superfamily. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MRP subfamily that is involved in multi-drug resistance. The encoded protein functions as a chloride channel and controls the regulation of other transport pathways. Mutations in this gene are associated with the autosomal recessive disorders cystic fibrosis and congenital bilateral aplasia of the vas deferens. Alternatively spliced transcript variants have been described, many of which result from mutations in this gene. [provided by RefSeq, Jul 2008]

Function:
Involved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter. Can inhibit the chloride channel activity of ANO1.

Subunit:
Interacts with SHANK2 (By similarity). Interacts with SLC9A3R1, MYO6 and GOPC. Interacts with SLC4A7 through SLC9A3R1. Found in a complex with MYO5B and RAB11A. Interacts with ANO1.

Subcellular Location:
Early endosome membrane; Multi-pass membrane protein. Cell membrane.

Tissue Specificity:
Found on the surface of the epithelial cells that line the lungs and other organs.

Post-translational modifications:
Phosphorylated; activates the channel. It is not clear whether PKC phosphorylation itself activates the channel or permits activation by phosphorylation at PKA sites. Phosphorylated by AMPK.
Ubiquitinated, leading to its degradation in the lysosome. Deubiquitination by USP10 in early endosomes, enhances its endocytic recycling.

DISEASE:
Cystic fibrosis (CF) [MIM:219700]: A common generalized disorder of the exocrine glands which impairs clearance of secretions in a variety of organs. It is characterized by the triad of chronic bronchopulmonary disease (with recurrent respiratory infections), pancreatic insufficiency (which leads to malabsorption and growth retardation) and elevated sweat electrolytes. It is the most common genetic disease in Caucasians, with a prevalence of about 1 in 2'000 live births. Inheritance is autosomal recessive. Note=The disease is caused by mutations affecting the gene represented in this entry.
Congenital bilateral absence of the vas deferens (CBAVD) [MIM:277180]: Important cause of sterility in men and could represent an incomplete form of cystic fibrosis, as the majority of men suffering from cystic fibrosis lack the vas deferens. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the ABC transporter superfamily. ABCC family. CFTR transporter (TC 3.A.1.202) subfamily.
Contains 2 ABC transmembrane type-1 domains.
Contains 2 ABC transporter domains.

SWISS:
P13569

Gene ID:
1080

Database links:

Entrez Gene: 1080 Human

Entrez Gene: 12638 Mouse

Omim: 602421 Human

SwissProt: P13569 Human

SwissProt: P26361 Mouse

Unigene: 489786 Human

Unigene: 621460 Human

Unigene: 661104 Human

Unigene: 15621 Mouse



CFTR是一種獨特的氯離子通道,盡管稱為氯離子通道,但在結(jié)構(gòu)上卻明顯地不同于其他氯離子通道,屬于一種ATP結(jié)合盒轉(zhuǎn)運體(ATP-binding cassettetransporter, ABC)家族。CFTR主要為氯離子跨上皮運動提供了選擇性通道,對于跨上皮的鹽類運輸、液體流動和離子濃度調(diào)節(jié)等都具有重要的決定作用。
CFTR分布廣泛,許多器官,如肺、肝、胰腺、腸、生殖腺等的細(xì)胞膜中都有表達,盡管稱為氯離子通道,但還涉及到其他一價陰離子的運輸,由于生理條件下氯離子最為重要,故稱為氯離子通道,近年來,它一直是離子通道研究中的一個熱點。
產(chǎn)品圖片
Tissue/cell: Rat lung tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-CFTR Polyclonal Antibody, Unconjugated(bs-1277R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: rat colon tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-CFTR Polyclonal Antibody, Unconjugated(bs-1277R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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