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CFTR (Cystic Fibrosis Transmembrane Conductance Regulator), Monoclonal Antibody

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产品名称: CFTR (Cystic Fibrosis Transmembrane Conductance Regulator), Monoclonal Antibody
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简单介绍

CFTR (Cystic Fibrosis Transmembrane Conductance Regulator), Monoclonal Antibody


CFTR (Cystic Fibrosis Transmembrane Conductance Regulator), Monoclonal Antibody  的详细介绍
Product Name

CFTR (Cystic Fibrosis Transmembrane Conductance Regulator), Monoclonal Antibody

Popular Item
Full Product Name

CFTR (Cystic Fibrosis Transmembrane Conductance Regulator)

Product Synonym Names
ABC35; ATP Binding Cassette Superfamily C Member 7 (ABCC7); cAMP-dependent chloride channel; CFTR; CFTR/MRP; Channel conductance-controlling ATPase; Cystic Fibrosis Transmembrane Conductance Regulator; MRP7; TNR CFTR
Product Gene Name

anti-CFTR antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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3D Structure
ModBase 3D Structure for P13569
Clonality
Monoclonal
Isotype
IgG2b
Clone Number
CFTR/1785
Host
Mouse
Species Reactivity
Human. Others not known.
Specificity
Recognizes a protein of 165-170kDa, identified as cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is composed of two membrane-spanning domains (MSD), two nucleotide-binding domains (NBD), and an R domain. It is structurally similar to multidrug resistance (Mdr1) protein and both are members of the superfamily of ATP-binding cassette (ABC) transporters, also known as traffic ATPases, which are implicated in the movement of various substrates. The CFTR protein is a small conductance adenosine 3', 5'-cyclic monophosphate (cAMP)-activated chloride ion channel found in the apical membranes of epithelia within the pancreas, airway, intestine, bile duct, sweat gland, and male genital ducts. CFTR is a valuable marker of human pancreatic duct cell development and differentiation.
Form/Format
200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.
Immunogen
Recombinant human CFTR fragment (aa258-385)
Cellular Localization
Cell Surface and Cytoplasmic
Hu-chromosome Location
7q31.2
Positive Control
MOLT-4 cells. Pancreas, Kidney or Placenta.
Preparation and Storage
Antibody with azide - store at 2 to 8 degree C.
Antibody without azide - store at -20 to -80 degree C.
Antibody is stable for 24 months. Non-hazardous. No MSDS required.
Other Notes
Small volumes of anti-CFTR antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Applications Tested/Suitable for anti-CFTR antibody
Immunofluorescence (IF), Immunohistochemistry (IHC) Formalin
Application Notes for anti-CFTR antibody
IF: 0.5-1ug/ml
IHC-F: 1-2ug/ml for 30 minutes at RT; Staining of formalin-fixed tissues is enhanced by boiling tissue sections in 10mM Tris with 1mM EDTA, pH 9.0 for 10-20 min followed by cooling at RT for 20 minutes.

Immunohistochemistry (IHC) of anti-CFTR antibody
Formalin-fixed, paraffin-embedded Human Pancreas stained with CFTR Monoclonal Antibody (CFTR/1785).
anti-CFTR antibody Immunohistochemistry (IHC) (IHC) image
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NCBI/Uniprot data below describe general gene information for CFTR. It may not necessarily be applicable to this product.
NCBI GI #
90421313
NCBI GeneID
1080
NCBI Accession #
NP_000483.3 [Other Products]
NCBI GenBank Nucleotide #
NM_000492.3 [Other Products]
UniProt Primary Accession #
P13569 [Other Products]
UniProt Secondary Accession #
Q20BG8; Q20BH2; Q2I0A1; Q2I102[Other Products]
UniProt Related Accession #
P13569[Other Products]
Molecular Weight
165-170kDa
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NCBI Official Full Name
cystic fibrosis transmembrane conductance regulator
NCBI Official Synonym Full Names
cystic fibrosis transmembrane conductance regulator
NCBI Official Symbol
CFTR  [Similar Products]
NCBI Official Synonym Symbols
CF; MRP7; ABC35; ABCC7; CFTR/MRP; TNR-CFTR; dJ760C5.1
  [Similar Products]
NCBI Protein Information
cystic fibrosis transmembrane conductance regulator
UniProt Protein Name
Cystic fibrosis transmembrane conductance regulator
UniProt Synonym Protein Names
ATP-binding cassette sub-family C member 7; Channel conductance-controlling ATPase (EC:3.6.3.49
Protein Family
Cystic fibrosis transmembrane conductance regulator
UniProt Gene Name
CFTR  [Similar Products]
UniProt Synonym Gene Names
ABCC7; CFTR  [Similar Products]
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NCBI Summary for CFTR
This gene encodes a member of the ATP-binding cassette (ABC) transporter superfamily. The encoded protein functions as a chloride channel, making it unique among members of this protein family, and controls ion and water secretion and absorption in epithelial tissues. Channel activation is mediated by cycles of regulatory domain phosphorylation, ATP-binding by the nucleotide-binding domains, and ATP hydrolysis. Mutations in this gene cause cystic fibrosis, the most common lethal genetic disorder in populations of Northern European descent. The most frequently occurring mutation in cystic fibrosis, DeltaF508, results in impaired folding and trafficking of the encoded protein. Multiple pseudogenes have been identified in the human genome. [provided by RefSeq, Aug 2017]
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UniProt Comments for CFTR
Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis (PubMed:26823428). Mediates the transport of chloride ions across the cell membrane (PubMed:10792060, PubMed:11524016, PubMed:11707463, PubMed:12519745, PubMed:15010471, PubMed:12588899, PubMed:17036051, PubMed:19398555, PubMed:19621064, PubMed:22178883, PubMed:25330774, PubMed:1712898, PubMed:8910473, PubMed:9804160, PubMed:12529365, PubMed:17182731, PubMed:26846474, PubMed:28087700). Channel activity is coupled to ATP hydrolysis (PubMed:8910473). The ion channel is also permeable to HCO3-; selectivity depends on the extracellular chloride concentration (PubMed:15010471, PubMed:19019741). Exerts its function also by modulating the activity of other ion channels and transporters (PubMed:12403779, PubMed:22178883, PubMed:22121115, PubMed:27941075). Plays an important role in airway fluid homeostasis (PubMed:16645176, PubMed:19621064, PubMed:26823428). Contributes to the regulation of the pH and the ion content of the airway surface fluid layer and thereby plays an important role in defense against pathogens (PubMed:14668433, PubMed:16645176, PubMed:26823428). Modulates the activity of the epithelial sodium channel (ENaC) complex, in part by regulating the cell surface expression of the ENaC complex (PubMed:17434346, PubMed:27941075, PubMed:17182731). Inhibits the activity of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G (PubMed:17182731). Inhibits the activity of the ENaC channel containing subunits SCNN1D, SCNN1B and SCNN1G, but not of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G (PubMed:27941075). May regulate bicarbonate secretion and salvage in epithelial cells by regulating the transporter SLC4A7 (PubMed:12403779). Can inhibit the chloride channel activity of ANO1 (PubMed:22178883). Plays a role in the chloride and bicarbonate homeostasis during sperm epididymal maturation and capacitation (PubMed:19923167, PubMed:27714810).
Product References and Citations for anti-CFTR antibody
s Riordan, J.R., et al. 1989. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245: 1066-1073.

Research Articles on CFTR
1. The pancreatic insufficiency (PI) status was associated with clinical variables and single nucleotide polymorphisms (SNPs) related with inflammatory response considering cystic fibrosis transmembrane conductance regulator (CFTR) mutations.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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