Page 5 - CF Roundtable - Winter/Spring 2026
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RESEARCH ROUNDUP (continued)
Diseases Common in Persons With Cystic Fibrosis
Among CFTR Heterozygotes
https://tinyurl.com/cy56wst7
CF is one of the most commonly diagnosed autosomal
recessive disorders in the US. It is estimated that more than
10 million individuals are heterozygous for a pathogenic
CFTR gene variant in the US (heterozygotes). The phenotypic
risk of these heterozygotes is not well defined, particularly
among populations of predominantly non-European
genetic ancestry. Understanding disease risk across each
population can improve management strategies for all.
The objective of this study was to examine associations
of diseases across the phenome with CFTR heterozygotes.
In this genetic association study, most heterozygotes
did not appear to have a substantially higher risk of CF–
associated diseases during their adulthood compared to
noncarriers. Additional studies are needed to investigate
the underlying factors for the elevated risk of respiratory
and infectious diseases in some heterozygotes.
Clarametyx Biosciences Announces Positive
Interim Analysis in Phase 2A Study Evaluating
CMTX-101 for Infections Associated With
Cystic Fibrosis
https://tinyurl.com/2kzkx8m2
Clarametyx announced in June that it is advancing a Phase
1b/2a clinical trial evaluating its novel immune-enabling
antibody therapy CMTX-101 to treat CF-associated
pulmonary infections, based on the DMC’s approval
to proceed following results of a pre-specified interim
analysis.
Revealing The Impact Of Pseudomonas Aeruginosa
Quorum Sensing Molecule 2’aminoacetophenone
On The Human Bronchial-Airway Epithelium And
Pulmonary Endothelium Using A Human Airway-On-
A-Chip
https://tinyurl.com/dmrkjwu
Pseudomonas aeruginosa (PA) causes severe respiratory
infections utilizing multiple virulence functions. The earlier
work of this team of researchers demonstrated that the
Pseudomonas aeruginosa quorum-sensing molecule
2′-aminoacetophenone (2-AA) reprograms host immune
and metabolic pathways, thereby facilitating the pathogen’s
long-term persistence.However, its specific impact on the
bronchial airway epithelium and pulmonary endothelium
remains unknown. To evaluate the spatiotemporal changes
in 2-AA within the human airway, considering endothelial
cells as the primary point of contact when the route of
lung infection is hematogenous, these researchers utilized
the airway-on-a-chip platform lined by polarized human
bronchial airway epithelium and pulmonary endothelium.
The effects of 2-AA on epithelial and endothelial primary
cells studied within a dynamic microphysiological
environment that mimics the human lung airway deepen
our understanding of this quorum sensing (QS) signaling
molecule. This study offers novel insights into the functions
and interactions of 2-AA, paving the way for innovative,
 Research Roundup continued on page 14
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