Matthias Salathe
University of Kansas, KS·Physiology
Cystic fibrosis (CF) is an inherited disease in which thick, dehydrated mucus clogs the airways. Many people with CF also develop diabetes, called cystic fibrosis-related diabetes (CFRD).
- Fields
Physiology
Medicine & Clinical Research
Pharmacology
- Topics
cystic fibrosis
CFTR modulators
elexacaftor/tezacaftor/ivacaftor
- Grants
- 1 known grant, through Mar 2027
What they work on
- Test whether high glucose, acting through the RAGE receptor, reduces BK potassium channel activity and drains airway surface liquid in cystic fibrosis bronchial epithelial cells grown in the lab.
- Determine whether low-dose metformin, by blocking RAGE signaling, restores BK channel function and improves elexacaftor/tezacaftor/ivacaftor rescue of CFTR, the faulty CF protein, under high glucose.
- Examine in living systems whether RAGE-driven ion channel dysfunction links abnormal glucose control to worse lung function in CF, and whether metformin counters it independent of blood sugar.
- Relate glucose swings measured by continuous monitoring in CF and CFRD patients to expression of LRRC26, a BK channel subunit needed for its function in airway cells.
More about the research
It affects about 20% of adolescents and over 40% of adults with CF, and it strongly predicts worse lung function. Newer drugs called CFTR modulators repair the faulty CF protein and can improve blood sugar control. Yet the team's clinic data show that patients with CFRD still lose lung function faster on these drugs than patients without diabetes. This suggests high blood sugar harms the airways in ways that normal glucose control alone may not fix.
Why it matters
Lung disease remains the main threat for people with cystic fibrosis. Adults with CF-related diabetes keep losing lung function even on modern modulator drugs. If low-dose metformin, an inexpensive and widely used diabetes medicine, protects the ion channels that keep airway mucus hydrated, it could become an added therapy to help preserve lung function in CF patients with abnormal glucose control.
Known grants
From NIH and NSF research grants. Labs often have other funding we can't see.
Closest labs
Xiangming Ji
Pennsylvania State University, PA·Physiology
Determine how metallothionein 1E (MT1E), a metal-binding protein that buffers oxidative stress, affects the function and survival of insulin-producing beta cells during the development of cystic fibrosis-related diabetes.
Martina Gentzsch
University of North Carolina at Chapel Hill, NC·Medicine & Clinical Research
Provide lab-grown cell models and mouse and rabbit models of cystic fibrosis to study how defects in CFTR, the salt-and-water channel protein, cause disease, and to test new drug targets and treatments.
Lucas Hoffman
University of Washington, WA·Medicine & Clinical Research·Early-career
Test whether highly effective CFTR modulator therapy changes the gut microbiome and fecal metabolites, including bile acids, of people with cystic fibrosis, using stool samples and clinical data from two national observational studies.
The school
University of Kansas: 26 labs on PI Pages, $546M a year on research, 351 research doctorates a year.
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