Related Experiment Video

Updated: Sep 28, 2025

Real-Time, Semi-Automated Fluorescent Measurement of the Airway Surface Liquid pH of Primary Human Airway Epithelial Cells
10:18

Real-Time, Semi-Automated Fluorescent Measurement of the Airway Surface Liquid pH of Primary Human Airway Epithelial Cells

Published on: June 13, 2019

7.6K

Club cell secretory protein and lung function in children with cystic fibrosis

Jing Zhai1, Mary J Emond2, Amber Spangenberg1

  • 1Asthma and Airway Disease Research Center, University of Arizona, Tucson, AZ, United States.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|April 3, 2022
PubMed

Insights

Low serum club cell secretory protein (CC16) levels are linked to worse lung function in children with cystic fibrosis (CF). These deficits, combined with inflammation, worsen CF lung disease severity.

Area of Science:

  • Pulmonary Medicine
  • Genetics
  • Immunology

Background:

  • Club cell secretory protein (CC16) has anti-inflammatory properties relevant to lung diseases.
  • Cystic Fibrosis (CF) is a genetic disorder characterized by progressive lung damage.
  • Investigating CC16's role in CF lung function is crucial for understanding disease progression.

Purpose of the Study:

  • To examine the relationship between serum CC16 levels and lung function in children with CF.
  • To determine if genetic variants influencing CC16 levels impact CF lung function.
  • To assess the association between CC16 deficits, inflammation, and CF lung disease severity.

Main Methods:

  • Longitudinal data from 260 children with CF (EPIC Study) were analyzed.
  • Serum CC16 levels and an inflammatory score were compared between adolescents with lowest (LLF) and highest (HLF) lung function.
  • Single nucleotide variants (SNVs) in SCGB1A1 and EHF-APIP loci were tested for association with CC16 levels and lung function decline (FEV1, FEV1/FVC) from ages 7-16.

Main Results:

  • Children with lower lung function (LLF group) had significantly lower CC16 levels and higher inflammatory scores compared to the HLF group.
  • The combination of low CC16 and high inflammation increased the likelihood of having the lowest lung function.
  • A specific SNV (rs3741240) associated with CC16 levels was also linked to a decline in FEV1/FVC and FEV1.

Conclusions:

  • Serum CC16 deficits are strongly associated with CF lung disease severity.
  • The impact of CC16 deficits on CF lung disease is additive to systemic inflammation.
  • The rs3741240 A allele may contribute to reduced CC16 levels and accelerated lung function decline in CF patients.
Abstract

Keywords:
Club cell secretory proteinCystic fibrosisLung function

More Related Videos

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study08:00

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study

Published on: April 11, 2018

10.8K
The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections06:57

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections

Published on: May 8, 2017

9.3K

Related Concept Videos

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,...
382
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...
231
Overview of Secretory Vesicles01:33

Overview of Secretory Vesicles

Secretory vesicles, also known as dense core vesicles (DCVs), are membrane-bound vesicles that transport secretory proteins, such as hormones or neurotransmitters. Regulated secretory vesicles transport proteins from the trans-Golgi network to the exterior of the cell. Proteins present in regulated secretory vesicles are required to be rapidly exocytosed in large amounts upon a specific stimulus.
Various proteins regulate the aggregation of molecules inside the secretory vesicles. Chromogranins...
8.7K
Chest Physiotherapy01:24

Chest Physiotherapy

Chest Physiotherapy (CPT) is a therapeutic technique used in respiratory care to improve ventilation, clear bronchial secretions, and enhance the efficiency of respiratory muscles. This therapy includes three primary procedures: postural drainage, percussion, and vibration. It can be performed on spontaneously breathing patients and those who are intubated and mechanically ventilated.
Purpose
CPT is primarily used for patients with excessive bronchial secretions who have difficulty clearing...
799
Chronic Obstructive Pulmonary Disease-II: Pathophysiology01:20

Chronic Obstructive Pulmonary Disease-II: Pathophysiology

Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
3.2K
Breathing01:05

Breathing

The process of breathing, inhaling and exhaling, involves the coordinated movement of the chest wall, the lungs, and the muscles that move them. Two muscle groups with important roles in breathing are the diaphragm, located directly below the lungs, and the intercostal muscles, which lie between the ribs. When the diaphragm contracts, it moves downward, increasing the volume of the thoracic cavity and creating more room for the lungs to expand. When the intercostal muscles contract, the ribs...
60.5K