CFTR,囊性纤维化跨膜转运调节因子抗体

产品编号HZ-1277R
英文名称CFTR
中文名称囊性纤维化跨膜转运调节因子抗体
别 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.
说 明 书0.1ml 0.2ml
CFTR,囊性纤维化跨膜转运调节因子抗体研究领域细胞生物 免疫学 新陈代谢
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep,
产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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168kDa
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human CFTR
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.
CFTR,囊性纤维化跨膜转运调节因子抗体PubMedPubMed
产品介绍background:
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.

CFTR,囊性纤维化跨膜转运调节因子抗体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.

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

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

CFTR是一种独特的氯离子通道，尽管称为氯离子通道，但在结构上却明显地不同于其他氯离子通道，属于一种ATP结合盒转运体(ATP-binding cassettetransporter, ABC)家族。CFTR主要为氯离子跨上皮运动提供了选择性通道，对于跨上皮的盐类运输、液体流动和离子浓度调节等都具有重要的决定作用。
CFTR分布广泛，许多器官，如肺、肝、胰腺、肠、生殖腺等的细胞膜中都有表达，尽管称为氯离子通道，但还涉及到其他一价阴离子的运输，由于生理条件下氯离子最为重要，故称为氯离子通道，近年来，它一直是离子通道研究中的一个热点。