Rabbit Anti-Human CFTR (a.a. 1203-1426) Polyclonal antibody

Name: Rabbit Anti-Human CFTR (a.a. 1203-1426) Polyclonal antibody
SKU: DPABH-25194
Rating: 5 (1 reviews)

Rabbit Anti-Human CFTR (a.a. 1203-1426) Polyclonal antibody for ELISA, IHC, WB

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COA

SDS

Datasheet

Key features and details

Target: Human CFTR
Host: Rabbit
Application: ELISA, IHC, WB
Species Reactivity: Human

Summary

Specifications

Antibody Isotype

IgG

Species Reactivity

Human

Immunogen

E. coli - derived recombinant Human CFTR (Ile1203-Ser1426).

Conjugate

Unconjugated

Applications

Application Notes

ELISA: 1:4000-1:8000
IHC: 1:50-1:100
WB: 1:1000-1:4000

General Notes

This product (Catalog No. DPABH-25194) is a rabbit-derived polyclonal antibody raised against human CFTR, targeting the amino acid region 1203–1426 of the protein. It has been affinity purified by Protein A. The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is a membrane protein functioning as an ion channel that transports chloride and bicarbonate ions across epithelial cell membranes. By regulating salt and water balance on surfaces like the lungs, pancreas, and sweat glands, it maintains thin, hydrated mucus. CFTR mutations cause cystic fibrosis, leading to thick mucus, infection, and inflammation.
DPABH-25194 is suitable for use in ELISA (1:4,000–1:8,000), IHC (1:50–1:100), Western blot (WB) (1:1,000–1:4,000) applications.
DPABH-25194 is supplied as a liquid formulation in 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. For long-term storage, keep at −20 °C. For short-term use, store at 2–8 °C. Avoid repeated freeze–thaw cycles to maintain antibody integrity and performance.

Target

Alternative Names

Cystic fibrosis transmembrane conductance regulator; CFTR; ATP-binding cassette sub-family C member 7; Channel conductance-controlling ATPase; 5.6.1.6; cAMP-dependent chloride channel; CFTR; ABCC7

UniProt ID

P13569

Citations

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Documents

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References

Evidence-based treatment of cystic fibrosis

INTERNIST

Authors: Ringshausen, F. C.; Hellmuth, T.; Dittrich, A. -M.

Abstract

Mucoviscidosis (cystic fibrosis [CF]) is the most common autosomal recessive inherited multisystem disease with fatal outcome. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which lead to a dysfunctional chloride channel and a defective CFTR protein. As a consequence, retention of insufficiently hydrated mucus affects multiple essential organs, mainly the lungs and airways, pancreas, liver, biliary tract and intestines. This leads to inflammation and infection, fibrosis and progressive tissue destruction. Respiratory failure is the major cause of mortality; however, in the no more than 30 years since the molecular characterization of the basic CFTR defect causing CF, tremendous success has been made with respect to the long-term prognosis of people with CF. This improvement in the prognosis was achieved by the cooperative spirit and networking of the very active and international CF research community and by establishing a multidisciplinary clinical CF team that implements the existing evidence in various aspects of standardized care together with the CF patient. This narrative review article presents the evidence in selected aspects of CF treatment, with special consideration of the most recent development of highly effective CFTR modulator treatment. This treatment will soon become available for more than 90% of the global CF patients and transform the pathophysiology as well as the course of disease towards a treatable chronic condition in internal medicine.

Insights into the variability of nasal potential difference, a biomarker of CFTR activity

JOURNAL OF CYSTIC FIBROSIS

Authors: Kyrilli, Spyridoula; Henry, Theophraste; Wilschanski, Michael; Fajac, Isabelle; Davies, Jane C.; Jais, Jean-Philippe; Sermet-Gaudelus, Isabelle

Abstract

Background: Nasal potential difference (NPD) is used to evaluate CFTR function in vivo. We aimed to evaluate the intrasubject and intersubject variability of NPD measurements. Methods: We reviewed NPD tracings of 116 patients with CF enrolled in the placebo arm of a multicenter study. Patients carried at least one nonsense mutation and underwent repeated NPD tests every 16 weeks. NPD parameters included basal potential difference (basal PD), inhibition of sodium absorption by amiloride (Delta Amiloride), chloride (Cl-) transport in response to a Cl--free solution (Delta Low Cl-), isoproterenol (Delta Isoproterenol), the sum of Delta Low Cl- and Delta Isoproterenol (Delta Low Cl--Isoproterenol) and ATP (Delta ATP). Results: Basal PD and Delta Amiloride displayed the highest variabilities, mainly stemming from intercenter and intrasubject effect. Delta Low Cl-, Delta Isoproterenol and Delta Low Cl--Isoproterenol demonstrated a large intrasubject variability but a smaller intersubject variability. The intrasubject measurement variability for Delta Low Cl--Isoproterenol, was within +/- 7.2 mV with 95% probability. It was greater in patients reporting ongoing pulmonary exacerbations. Conclusions: The large intercenter variability of basal PD and Delta Amiloride highlights the operatordependent aspect of these measurements. A difference greater than 7.2 mV in Delta Low Cl--Isoproterenol in a given patient on CFTR modulator can be attributed, with 95% probability, to a treatment effect rather than to the variability inherent in the measurement. (C) 2019 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.