Cystic Fibrosis Research Translation Center
The primary goal of the Cystic Fibrosis Research and Translation Center (CFRTC) is to be a world leader in clinical-translational science in cystic fibrosis (CF), spanning from early drug development to dissemination into the CF community. Through the strength of the Microbiology, Genomics, Host Response and Clinical Cores as well as the pilot program, we strive to understand the bacterial-host interactions in the pulmonary and gastrointestinal tracts of patients with CF. We focus on interdisciplinary studies and encourage new investigators in digestive diseases, nutrition and metabolism, and inflammation. To help reach this important goal, we plan to implement the following four aims:
- Provide resources and expertise to expedite development of promising new therapeutic approaches to correct dysfunctional CFTR and its secondary consequences including maldigestion and malnutrition, hepatobiliary disease and chronic lung disease.
- Foster collaboration across P30 biomedical cores and enhance translational research nationally through sharing of human and bacterial repositories linked to clinical databases. Utilize the shared specimen and data resources to develop improved assays, new drug screening assays, biomarkers and improved clinical outcome measures.
- Enhance understanding of evolving bacterial ecosystems and resultant host response in CF gastrointestinal and respiratory tracts, and how these interactions impact health.
- Better understand the metabolic and inflammatory consequences of CFTR dysfunction, including probing whether CFTR modulators impact CFTR dysfunction, thereby leading to pro-inflammatory changes in immune cells in patients with CF.
Cystic Fibrosis Research Development Program
The University of Washington’s three-decade history preforming clinical, basic and translational CF research originated from the CFF RDP, and the RDP remains its foundation. Our RDP has developed new CF treatments that are in use worldwide. RDP investigators have also conceived of novel therapeutics that are currently at various stages of development from early laboratory work, to phase 2 clinical trials. The RDP has generated extensive new knowledge about CF infections, CF lung and intestinal disease, and the basic biology of CF pathogens. The scientific and human resources of the RDP have given rise to the Therapeutics Development Network Coordinating Center, two CF center-wide NIH P30 grants, and many individual research awards from NIH and other agencies. The RDP has trained generations of CF researchers and clinicians, including innovative physician-scientists committed to CF research over the duration of their careers, and leaders in CF research and care at other institutions. The RDP also developed internationally-valued resources such as isolate collections, mutant libraries, genomics resources, and research tools utilized by hundreds of investigators worldwide. The broad objectives of our RDP are to:
- Generate and disseminate new scientific knowledge in our four established and three emerging focus areas.
- Foster collaboration among CF researchers.
- Bring new and young faculty investigators into CF research.
- Enhance CF-focused translational, basic and clinical post-doctoral training.
- Leverage RDP program resources to obtain additional extramural funding for CF research.
- Advance innovative therapies to clinical application to improve the health of CF patients.
Supported by a grants from the National Institutes of Health (NIDDK P30 DK 089507) and the Cystic Fibrosis Foundation (SINGH15R0). Please reference these grant numbers on all publications resulting from support provided by these Core Center grants.
Matamouros S, et al. Adaptation of commensal proliferating Escherichia coli to the intestinal tract of young children with cystic fibrosis. Proc Natl Acad Sci U S A. 2018 Jan 29.
Adaptation of commensal proliferating Escherichia coli to the intestinal tract of young children with cystic fibrosis
Matamouros S, Hayden HS, Hager KR, Brittnacher MJ, Lachance K, Weiss EJ, Pope CE, Imhaus AF, McNally CP, Borenstein E, Hoffman LR, Miller SI.
Gelfond, et al. Pancreatic Enzyme Replacement Therapy use in infants with Cystic Fibrosis Diagnosed by Newborn Screening. J Pediatr Gastroenterol Nutr. 2017 Nov 15.
Pancreatic Enzyme Replacement Therapy use in infants with Cystic Fibrosis Diagnosed by Newborn Screening
Gelfond, Daniel; Heltshe, Sonya L; Skalland, Michelle; Heubi, James E; Kloster, Margaret; Leung, Daniel H; Ramsey, Bonnie W; Borowitz, Drucy; BONUS Study Investigators.
J Pediatr Gastroenterol Nutr.
Toussaint, et al. Gene Duplication in Pseudomonas aeruginosa Improves Growth on Adenosine. J Bacteriol. 2017 Nov 01.
Toussaint, Jean-Paul; Farrell-Sherman, Anna; Feldman, Tamar Perla; Smalley, Nicole E; Schaefer, Amy L; Greenberg, E Peter; Dandekar, Ajai A
J Bacteriol. 2017 Oct 3;199(21). pii: e00261-17. doi: 10.1128/JB.00261-17. Print 2017 Nov 1.
Heltshe, et al. Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis. J Cyst Fibros. 2017 Oct 27.
Longitudinal development of initial, chronic and mucoid Pseudomonas aeruginosa infection in young children with cystic fibrosis
Heltshe, S L; Khan, U; Beckett, V; Baines, A; Emerson, J; Sanders, D B; Gibson, R L; Morgan, W; Rosenfeld, M
J Cyst Fibros.
Hisert, et al. Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections. Am J Respir Crit Care Med. 2017 Jun 15.
Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections
Hisert, Katherine B; Heltshe, Sonya L; Pope, Christopher; Jorth, Peter; Wu, Xia; Edwards, Rachael M; Radey, Matthew; Accurso, Frank J; Wolter, Daniel J; Cooke, Gordon; Adam, Ryan J; Carter, Suzanne; Grogan, Brenda; Launspach, Janice L; Donnelly, Seamas C; Gallagher, Charles G; Bruce, James E; Stoltz, David A; Welsh, Michael J; Hoffman, Lucas R; McKone, Edward F; Singh, Pradeep K.
Stephenson, et al. Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States: A Population-Based Cohort Study Ann Intern Med. 2017 Mar 14
Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States: A Population-Based Cohort Study
Stephenson AL, Sykes J, Stanojevic S, Quon BS, Marshall BC, Petren K, Ostrenga J, Fink AK, Elbert A, Goss CH.
Ann Intern Med.
In 2013, the ATS Foundation and the ATS Assembly on Pediatrics established the Robert B. Mellins, MD, Outstanding Achievement Endowed Award honoring Dr. Mellins’ legacy as a founder of pediatric pulmonology. The Mellins Award recognizes the scholarship and research achievement of an instructor, assistant professor, or early associate professor in the pediatric pulmonology field.
Dr. Bonnie W. Ramsey has been elected to membership in the National Academy of Medicine.
Ramsey is a leader in cystic fibrosis clinical care and research and a UW Medicine and Seattle Children’s pediatrician specializing in lung diseases.
The Shaw Prize in Life Science and Medicine is awarded in equal shares to
E Peter Greenberg Professor of Microbiology, University of Washington, USA and
Bonnie L Bassler Squibb Professor and Chair, Department of Molecular Biology, Princeton University and Investigator of the Howard Hughes Medical Institute, USA
for their discovery of quorum sensing, a process whereby bacteria communicate with each other and which offers innovative ways to interfere with bacterial pathogens or to modulate the microbiome for health applications.
Cystic Fibrosis Research and Translation Center
Seattle Children’s Research Institute
2001 8th Ave
Seattle, WA 98121