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Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

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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,...
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Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

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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...
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Mutations01:39

Mutations

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Overview
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Vesicular Tubular Clusters01:45

Vesicular Tubular Clusters

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After budding out from the ER membrane, some COPII vesicles lose their coat and fuse with one another to form larger vesicles and interconnected tubules called vesicular tubular clusters or VTCs. These clusters constitute a compartment at the ER-Golgi interface known as ERGIC (Endoplasmic Reticulum Golgi Intermediate Compartment). The ERGIC is a mobile membrane-bound cargo transport system that sorts proteins secreted from ER and delivers them to the Golgi.
With the help of motor proteins such...
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Transcellular Transport of Solutes01:23

Transcellular Transport of Solutes

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Transcellular transport of solutes is the movement of substances like monosaccharides and amino acids through polarized cells. This transport mechanism is primarily seen in epithelial and endothelial cells aided by membrane transport proteins such as channels and transporters. The tight junctions between these cells confine the membrane proteins to the two sides of the cell. The epithelial cells have distinct apical and basolateral domains. In contrast, the endothelial cells show the luminal...
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Transport Across the Golgi01:26

Transport Across the Golgi

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While it is unclear how molecules move between adjacent Golgi cisternae, it is apparent that the molecules move from cis- cisterna, the entry face, to the trans- cisterna, the exit face. Experiments initially suggested vesicles that bud from one cisterna and fuse with the next cisterna to transport proteins between the cisternae. This vesicular transport model describes the Golgi apparatus as a relatively static structure with a unique enzyme composition in each cisterna. Molecules are...
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Related Experiment Video

Updated: Apr 28, 2026

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

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CFTR: cystic fibrosis and beyond.

Marcus A Mall1, Dominik Hartl2

  • 1Dept of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), University of Heidelberg, Member of the German Center for Lung Research (DZL), Heidelberg, Germany Division of Paediatric Pulmonology and Allergy and Cystic Fibrosis Center, Dept of Paediatrics, University of Heidelberg, Member of the German Center for Lung Research (DZL), Heidelberg, Germany Marcus.Mall@med.uni-heidelberg.de.

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Summary

Cystic fibrosis (CF) is a fatal lung disease. Advances in understanding CFTR gene function have led to new therapies for some patients, with efforts underway to help more individuals with CF.

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Area of Science:

  • Pulmonary Medicine
  • Genetics
  • Molecular Biology

Background:

  • Cystic fibrosis (CF) is the most prevalent fatal hereditary lung disorder.
  • The discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene has been pivotal.
  • This led to understanding CF lung disease mechanisms, developing animal models, and creating mutation-specific therapies.

Discussion:

  • CFTR dysfunction causes mucus obstruction, inflammation, and infection in CF airways.
  • Recent breakthroughs and challenges in therapies targeting the basic CF defect are discussed.
  • The article examines strategies to extend disease-modifying therapies to more CF patients, including those with the common ΔF508-CFTR mutation.

Key Insights:

  • Understanding CFTR gene function is crucial for CF pathogenesis.
  • Mutation-specific therapies are emerging but accessibility remains a challenge.
  • CFTR dysfunction's role in CF lung disease is complex, involving mucus, inflammation, and infection.

Outlook:

  • Future efforts focus on making disease-modifying therapies available to a larger CF patient population.
  • Investigating acquired CFTR dysfunction in other lung diseases like COPD is a promising area.
  • Lessons from CF research may inform treatments for common airway diseases with mucus plugging.