Xiangming Ji
Pennsylvania State University, PA·Physiology
Cystic fibrosis (CF) is an inherited disease caused by mutations in CFTR, a gene whose protein moves chloride across cell membranes; losing it leaves thick secretions that damage the lungs and digestive organs. About half of adults with CF also develop cystic fibrosis-related diabetes (CFRD), which starts far earlier in life than typical type 1 or type 2 diabetes and makes lung disease considerably worse.
- Fields
Physiology
Medicine & Clinical Research
Biochemistry
- Topics
cystic fibrosis
CFTR modulators
metallothionein 1E
- Grants
- 1 known grant, through May 2029
What they work on
- 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.
- Test whether pancreatic digestive (exocrine) cells expressing mutant CFTR damage neighboring hormone-producing endocrine islets, using human exocrine and endocrine pancreatic organoids grown from pluripotent stem cells.
- Map where and how severely fibrosis develops in the pancreas as cystic fibrosis-related diabetes progresses, using collagen-targeted magnetic resonance imaging.
- Build a single-cell RNA sequencing map of pancreatic islets from healthy, CFRD and TRIKAFTA-treated CFTR G551D ferrets to identify biomarkers of disease progression.
More about the research
New CFTR-correcting drugs such as the triple-combination therapy TRIKAFTA improve lung function but do not appear to fix the hormone-producing failures of the pancreas. Why the insulin-producing cells falter, and how to detect the problem early, are open questions.
Why it matters
People with cystic fibrosis frequently develop diabetes in childhood, and it worsens their lung disease and shortens life, yet current CFTR-modulator drugs do not seem to prevent it. Clarifying how MT1E and pancreatic scarring contribute could reveal treatment targets, and collagen-targeted MRI could become a way to detect pancreatic damage early, before diabetes is established, allowing earlier management for people with CF.
Known grants
From NIH and NSF research grants. Labs often have other funding we can't see.
Closest labs
Matthias Salathe
University of Kansas, KS·Physiology
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.
Barak Blum
University of Wisconsin-Madison, WI·Physiology
Test the hypothesis that reducing Robo2 receptors in beta cells, the pancreas's insulin-making cells, is essential for islets of Langerhans to expand successfully when insulin demand rises.
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.
The school
Pennsylvania State University: 60 labs on PI Pages, $1.3B a year on research, 743 research doctorates a year.
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