Texas [US], September 27 : Five lung stem cell variants predominate in the lungs of patients with advanced cystic fibrosis (CF), Wa Xian and Frank McKeon, two nationally renowned experts in cloning and stem cell science from the University of Houston report. These variants are responsible for important CF pathology features like inflammation, fibrosis, and mucin secretion.
The genetic and progressive condition cystic fibrosis impairs breathing and leads to recurrent lung infections. The cystic fibrosis transmembrane conductance regulator (CFTR) gene deficiency is the source of this condition, which affects close to 40,000 persons in the US. Chronic lung illness, characterised by excessively thick, sticky, and sticky mucus that clogs organs, primarily the lungs, is brought on by CFTR gene defects.
Recently introduced drugs known as CFTR modulators act to rescue the function to the mutant CFTR gene and yield remarkable improvements in lung function of CF patients. However, in patients with established lung disease, lung inflammation remains despite treatment with CFTR modulators. This persistence is concerning as inflammation is thought to be a key factor in the progression of CF lung disease.
This gap in CFTR modulator efficacy renders the work of the Xian-McKeon laboratory particularly relevant.
“Using single cell cloning technology that detailed stem cell heterogeneity in lungs from patients with COPD and idiopathic pulmonary fibrosis (IPF), we identified five stem cell variants common to lungs of patients with advanced CF, including three that show hyperinflammatory gene expression profiles and drive neutrophilic inflammation upon xenografting to immunodeficient mice,” said Xian, research professor in biology and biochemistry.
“We found that CFTR-modulating drugs did not suppress the proinflammatory activity or gene expression of the three CF variants that drive inflammation,” reports McKeon, professor of biology and biochemistry and director of the Stem Cell Center, in the American Journal of Respiratory and Critical Care Medicine. “These findings raise the possibility that these inflammatory stem cell variants are the source of the persistent inflammation in patients treated with CFTR modulators.”
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