Related Experiment Video

Updated: May 5, 2026

In Vitro Analysis of PDZ-dependent CFTR Macromolecular Signaling Complexes
10:05

In Vitro Analysis of PDZ-dependent CFTR Macromolecular Signaling Complexes

Published on: August 13, 2012

10.9K

The ABC protein turned chloride channel whose failure causes cystic fibrosis

David C Gadsby1, Paola Vergani, László Csanády

  • 1Laboratory of Cardiac/Membrane Physiology, The Rockefeller University, New York, NY 10021, USA. gadsby@rockefeller.edu

Nature
|March 24, 2006
PubMed

Insights

Cystic fibrosis transmembrane conductance regulator (CFTR) channels, a type of ABC ATPase, use ATP to gate anion flow. New insights reveal a unifying mechanism for ATP-driven conformational changes in CFTR and related proteins.

Area of Science:

  • Biochemistry
  • Molecular Biology
  • Structural Biology

Background:

  • Cystic fibrosis transmembrane conductance regulator (CFTR) channels are ATP-binding cassette (ABC) transporters.
  • CFTR mutations cause cystic fibrosis, a genetic disorder.
  • CFTR channels regulate anion transport across cell membranes.

Purpose of the Study:

  • To elucidate the mechanism of ATP-driven conformational changes in CFTR channels.
  • To unify understanding of ABC transporter function.

Main Methods:

  • Analysis of structural and biochemical data from prokaryotic ABC proteins.
  • Integration of functional data from CFTR channels.

Main Results:

  • A unifying mechanism explaining ATP-driven conformational changes in ABC transporters, including CFTR, has been proposed.
  • This mechanism highlights the role of ATP in gating anion flow.

Conclusions:

  • The study provides a unified mechanistic framework for understanding CFTR and other ABC transporter functions.
  • This research advances knowledge of ion channel gating and ATP-driven molecular machines.

More Related Videos

Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae15:12

Purification of the Cystic Fibrosis Transmembrane Conductance Regulator Protein Expressed in Saccharomyces cerevisiae

Published on: May 10, 2014

14.7K
Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein09:59

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

Published on: March 9, 2015

11.7K

Related Concept Videos

Protein Complexes with Interchangeable Parts01:57

Protein Complexes with Interchangeable Parts

Groups of proteins may form a complex where each protein in this complex has a different role in the overall execution of the complex’s function. Often some of the proteins in the complex can be replaced by a closely related variant to give a complex that contains many of the same components yet is functionally distinct.
The SCF ubiquitin ligase is a protein complex of five individual proteins. This complex attaches ubiquitin to other target proteins to mark them for degradation. In order...
2.1K
ABC Transporters: Exporter01:31

ABC Transporters: Exporter

ATP-binding cassette or ABC transporter is the largest superfamily of integral membrane proteins. The transporters have transmembrane-binding domains (TMDs) and nucleotide-binding domains (NBDs). The TMDs are specific to their substrates, whereas the NBDs are similar to engines that complete ATP hydrolysis to complete the substrate transport. They can be full transporters consisting of two TMDs and NBDs, half transporters with one TMD and NBD, while some encoded with a single TMD or NBD are...
5.7K
ABC Transporters: Importer01:27

ABC Transporters: Importer

ATP-binding cassette or ABC transporters are a class of ATP-driven pumps that hydrolyze ATP to move solutes across the membrane. They can be grouped into importers and exporters. While exporters are present in all domains of life, importers exist only in bacteria and some plants.
In bacteria, based on the number of transmembrane helices and the chemical nature of their substrates, the ABC importers can be divided into three types:
2.7K
Membrane Asymmetry Regulating Transporters01:19

Membrane Asymmetry Regulating Transporters

Enzymes like flippase, floppase, and scramblase transfer phospholipids from one layer to another in the membrane, thereby affecting membrane asymmetry.
Flippase
Eukaryotic flippases are type-IV P-type ATPases or P4-ATPases belonging to P-type ATPase family proteins that are membrane-bound pumps involved in the ATP-mediated transport of ions and molecules across the membrane. Flippases flip specific phospholipids from the outer to the inner leaflet of a membrane. All P4-ATPases have one...
6.1K
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
1.1K
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
657