CWRU receives $2.1 million NIH grant to expand cystic fibrosis research models

July 26, 2011

CLEVELAND - July 26, 2011 -Case Western Reserve University School of Medicine has received a $2.1 million grant from the National Center for Research Resources, part of the National Institutes of Health (NIH), to expand basic research models for the study of cystic fibrosis (CF).

CF is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. The four-year NIH grant was awarded to Mitchell Drumm, PhD, and Craig Hodges, PhD, co-investigators of the research supported by the grant.

Drumm is professor of Pediatrics and Genetics at the Case Western Reserve School of Medicine, and vice chair for research in Pediatrics. He co-discovered the gene that causes CF, CFTR, along with Francis Collins, MD, PhD, the noted physician-geneticist and current director of the NIH. Hodges is an assistant professor of Pediatrics and Genetics at the Case Western Reserve School of Medicine as well.

Together, Drumm and Hodges have developed and studied a series of basic research models that have contributed significantly to the research community's understanding of CF and examined the effects of correcting the genetic defect in CF using these models. The newly secured grant provides the researchers the resources necessary to advance CF research through the development of additional basic research models.

"Basic research is critical in helping investigators pinpoint the cellular and molecular mechanisms that cause CF in order to determine how they can be reversed," Drumm said. "As there are currently a number of therapeutic approaches designed to correct CF gene function, it is important to understand what aspects of the disease can be corrected, or even reversed."

Such research entails the study of mouse genetics to better understand CF. Drumm and Hodges co-direct an animal core facility with the most comprehensive collection of CF mouse models in the world. With the new NIH grant, Drumm and Hodges plan to generate even more of these basic research models.

The models developed thus far include mice with different versions of the CF gene, each with a different amount of function, so that researchers can determine the critical levels of the gene function for the manifestation of any disease-related characteristic.

Recently, these researchers generated models that allow them to turn the CF gene on or off in different organs of the body, or at different times in the life of the animal. Turning the gene off enables researchers to determine how the gene directly and indirectly affects different organs, while turning it on allows researchers to assess the effects of correcting the CF gene. Their work with these mice has shown that the effects of the disease on the lungs and digestive system are more complex than originally thought, involving not only the cells lining those organs, but also the immune system and other types of cells. One of the apparent ramifications is that there may be targets for therapy not previously considered.

"Our hope is that we'll find out where in the body and when we need to focus our attention for therapies. This is a devastating disease for the children affected by it, and for their families, so anything we might do to improve the health of these kids would be of great significance."
-end-
About Case Western Reserve University School of Medicine

Founded in 1843, Case Western Reserve University School of Medicine is the largest medical research institution in Ohio and is among the nation's top medical schools for research funding from the National Institutes of Health. The School of Medicine is recognized throughout the international medical community for outstanding achievements in teaching. The School's innovative and pioneering Western Reserve2 curriculum interweaves four themes--research and scholarship, clinical mastery, leadership, and civic professionalism--to prepare students for the practice of evidence-based medicine in the rapidly changing health care environment of the 21st century. Eleven Nobel Laureates have been affiliated with the school.

Annually, the School of Medicine trains more than 800 MD and MD/PhD students and ranks in the top 25 among U.S. research-oriented medical schools as designated by U.S. News & World Report "Guide to Graduate Education."

The School of Medicine's primary affiliate is University Hospitals Case Medical Center and is additionally affiliated with MetroHealth Medical Center, the Louis Stokes Cleveland Department of Veterans Affairs Medical Center, and the Cleveland Clinic, with which it established the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University in 2002. http://casemed.case.edu.

Case Western Reserve University

Related Cystic Fibrosis Articles from Brightsurf:

Treating cystic fibrosis with mRNA therapy or CRISPR
The potential for treating cystic fibrosis (CF) using mRNA therapies or CRISPR gene editing is possible regardless of the causative mutation.

Cystic fibrosis: why so many respiratory complications?
Cystic fibrosis, one of the most common genetic diseases in Switzerland, causes severe respiratory and digestive disorders.

A newly discovered disease may lead to better treatment of cystic fibrosis
Cystic fibrosis is the most frequent severe inherited disorder worldwide.

New treatment kills off infection that can be deadly to cystic fibrosis patients
The findings, which are published in the journal Scientific Reports, show that scientists from Aston University, Mycobacterial Research Group, combined doses of three antibiotics -- amoxicillin and imipenem-relebactam and found it was 100% effective in killing off the infection which is usually extremely difficult to treat in patients with cystic fibrosis.

Cystic fibrosis carriers are at increased risk for cystic fibrosis-related conditions
A University of Iowa study challenges the conventional wisdom that having just one mutated copy of the cystic fibrosis (CF) gene has no effects on a person's health.

Rare mutations drive cystic fibrosis in Caribbean
Cystic Fibrosis (CF) in the Caribbean is dominated by unusual gene mutations not often observed in previously studied CF populations, according to comprehensive genome sequencing led by physician-scientists at UC San Francisco and Centro de Neumología Pediátrica in San Juan.

Cystic fibrosis carriers at increased risk of digestive symptoms
Researchers have found that carriers of the most common genetic variant that causes cystic fibrosis experience some symptoms similar to those of people with cystic fibrosis.

In cystic fibrosis, lungs feed deadly bacteria
A steady supply of its favorite food helps a deadly bacterium thrive in the lungs of people with cystic fibrosis, according to a new study by Columbia researchers.

Cibio knocks out cystic fibrosis
The fight against cystic fibrosis continues, targeting in particular some of the mutations that cause it.

Hypertonic saline may help babies with cystic fibrosis breathe better
Babies with cystic fibrosis may breathe better by inhaling hypertonic saline, according to a randomized controlled trial conducted in Germany and published in the American Thoracic Society's American Journal of Respiratory and Critical Care Medicine.

Read More: Cystic Fibrosis News and Cystic Fibrosis Current Events
Brightsurf.com is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to Amazon.com.