Thermo Fisher Scientific CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Recombinant Rabbit Monoclonal Antibody (CFTR/7154R)

Applications

Tested Dilution

Publications

Immunohistochemistry (Paraffin) (IHC (P))

1-2 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG, kappa

Expression System

HEK293 cells

Class

Recombinant Monoclonal

Type

Antibody

Clone

CFTR/7154R

Immunogen

Recombinant fragment (around aa 258-385) of human CFTR protein. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:CFTR,uniProtId:P13569-1,ncbiNodeId:9606,antigenRange:258-385,antigenLength:1480,antigenImageFileName:1080-RBM14-P1_CFTR_P13569-1_Rabbit.svg,antigenImageFileNamePDP:1080-RBM14-P1_CFTR_P13569-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

200 µg/mL

Purification

Protein A/G

Storage buffer

PBS with 0.05% BSA

Contains

0.05% sodium azide

Storage conditions

4° C

Shipping conditions

Ambient (domestic); Wet ice (international)

Product Specific Information

Antibody is stable for 24 months.

Positive Control: Human pancreas, kidney or placenta. Cellular Localization: Cell surface. Cytoplasm.

Specificity Comments: 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-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.

Target Information

Cystic Fibrosis (CF) is a common lethal genetic disease caused by mutations of the gene coding for the cystic fibrosis transmembrane conductance factor, a cAMP regulated chloride channel. Approximately 70% of all CF cases share the deletion of a phenylalanine at position 508 (delta F508) which results in abnormal chloride transport. Since the CF mutation is lethal, most often by lung and liver disease, it raises the question of why this genetic disease remains as common as it is. One possible explanation is that Salmonella typhi has been shown to use CFTR to enter intestinal epithelial cells and that delta F508 heterozygote and homozygote mice showed 86% and 100% reductions in S. typhi intestinal submucosal uptake.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.