Cftr Assay

A CFTR assay is a laboratory test that measures the abundance, localization, or function of the cystic fibrosis transmembrane conductance regulator, a chloride channel central to epithelial fluid balance and cystic fibrosis. In functional assays, cellular cAMP stimulation activates CFTR, allowing chloride movement across the membrane; researchers quantify this response with electrical, fluorescent, or other ion-sensitive readouts. Assay formats can distinguish defective channel activity from reduced protein expression or trafficking. In medicine, CFTR assays support cystic fibrosis diagnosis and disease research, help classify disease-associated variants, and evaluate CFTR modulators that aim to restore channel function for precision treatment.

Cftr Assay - Related Videos

Research

JoVE Journal - Biology

In Vitro Analysis of PDZ-dependent CFTR Macromolecular Signaling Complexes

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Cited by 7 •

2012

Cystic fibrosis transmembrane conductance regulator (CFTR), an epithelial chloride channel, has been reported to interact with various proteins and regulate important cellular processes; among them the CFTR PDZ motif-mediated interactions have been well documented. This protocol describes methods we developed to assemble a PDZ-dependent CFTR macromolecular signaling complex in vitro.

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein

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Cited by 7 •

2015

Described here is a rapid and effective procedure for functional reconstitution of purified wild-type and mutant CFTR protein that preserves activity for this chloride channel, which is defective in Cystic Fibrosis. Iodide efflux from reconstituted proteoliposomes mediated by CFTR allows studies of channel activity and the effects of small molecules.

Education

JoVE Science Education - Advanced Biology
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Cell-surface Biotinylation Assay

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2023

A cell can regulate the amount of particular proteins on its cell membrane through endocytosis, following which cell surface proteins are effectively sequestered in the cytoplasm. Once within a cell, these surface proteins can be either destroyed or “recycled” back to the membrane. The cell surface biotinylation assay provides researchers with a way to study these phenomena. The technique makes use of a derivative of the small molecule biotin, which can label surface proteins and then be...

Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients

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Cited by 121 •

2017

This protocol describes an assay for measuring CFTR function and CFTR modulator responses in cultured tissue from subjects with cystic fibrosis (CF). Biopsy-derived intestinal organoids swell in a cAMP-driven fashion, a response that is defective (or strongly reduced) in CF organoids and can be restored by exposure to CFTR modulators.

The TUNEL Assay

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2023

One of the hallmarks of apoptosis is the nuclear DNA fragmentation by nucleases. These enzymes are activated by caspases, the family of proteins that execute the cell death program. TUNEL assay is a method that takes advantage of this feature to detect apoptotic cells. In this assay, an enzyme called terminal deoxynucleotidyl transferase catalyzes the addition of dUTP nucleotides to the free 3’ ends of fragmented DNA. By using dUTPs that are labeled with chemical tags that can produce...

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