by The Scripps Research Institute Scripps researchers analyzed many genetic variants associated with cystic fibrosis across the population to determine how the CFTR protein’s functional structure changes with disease and therapeutics. Credit: Scripps Research To cure rare genetic diseases, from cystic fibrosis to Niemann-Pick, scientists at Scripps Research have turned to a computational approach usually...
UNIVERSITY OF HOUSTON IMAGE: WA XIAN AND FRANK MCKEON IN THE DEPARTMENT OF BIOLOGY AND BIOCHEMISTRY AND THE STEM CELL CENTER AT UNIVERSITY OF HOUSTON, ARE EXAMINING PRO-INFLAMMATORY STEM CELL VARIANTS IN CYSTIC FIBROSIS AND DEVELOPING THERAPEUTIC COMBINATIONS THAT SELECTIVELY TARGET THEM IN THE CF LUNG, WHILE SPARING THE NORMAL CELLS NEEDED FOR REGENERATIVE REPAIR....
UNIVERSITÉ DE GENÈVE IMAGE: HYDRATION RESTORES THE AIRWAY SURFACE SEAL. IN TURQUOISE, THE JUNCTIONS BETWEEN THE EPITHELIAL CELLS (BLUE). CREDIT: UNIGE – LABORATORY OF PROF. MARC CHANSON Cystic fibrosis is a rare genetic disease which can cause very serious symptoms. In particular, patients suffer from chronic bacterial infections that can lead to respiratory failure. It...
ANN & ROBERT H. LURIE CHILDREN’S HOSPITAL OF CHICAGO The lower airways of patients with cystic fibrosis (CF) have unique biochemical features that correlate with the complex communities of lung bacteria typical of this disease, according to a multicenter study led by researchers from Ann & Robert H. Lurie Children’s Hospital of Chicago. These findings...
UNIVERSITY OF TEXAS M. D. ANDERSON CANCER CENTER HOUSTON ― A multicenter research team co-led by The University of Texas MD Anderson Cancer Center developed the first drug to treat the uncontrolled secretion of mucins in the airways, which causes potentially life-threatening symptoms in millions of Americans with asthma, chronic obstructive pulmonary disease (COPD) and cystic fibrosis...
by University of Toronto “Clubbing” of the fingers is a classic features of Cystic Fibrosis, although not present in many patients. Credit: Jerry Nick, M.D./ Wikipedia Researchers at the University of Toronto have identified hundreds of new proteins that could play a role in cystic fibrosis, and which may shed light on why some patients...
by Cold Spring Harbor Laboratory Credit: Pixabay/CC0 Public Domain The process of generating proteins from genes is akin to a factory, where workers follow a set of instructions that, ideally, are effective and clear. But for some people who suffer from cystic fibrosis (CF), one of their genes has a mutation that results in confusing...
Protein misfolding is a likely culprit in many degenerative disorders. Cystic fibrosis, for instance, is caused by mutations in the CFTR gene that prevent the eponymous protein from assuming its proper configuration. Mutations that impair how other proteins fold have been linked to Alzheimer’s, Parkinson’s, and Huntington’s disease. Now, a new study demonstrates that drugs...
by La Jolla Institute for Immunology A partial bone marrow transplant helps these mice by introducing a population of healthy immune cells called monocytes. Credit: La Jolla Institute for Immunology Scientists at La Jolla Institute for Immunology (LJI) have found they can dramatically improve survival of mice with cystic fibrosis through a partial bone marrow...
Explaining how M. abscessus was ready once CF patients started living longer. Smoking can really clog up the lungs, even for people who’ve never been near a cigarette. Turns out that smoking habits from the early 1900s are still inflicting damage—not on tobacco users or their families, but on people with cystic fibrosis. Cystic fibrosis...
