 |
|
New links in the cystic fibrosis chain uncover potential therapeutics
October 19, 2007Cystic fibrosis (CF) is an inherited disease caused by mutations in the CFTR gene. Each mutation has number of effects on the cells of the lungs. New evidence generated in vitro by Vojo Deretic and colleagues at the University of New Mexico School of Medicine, Albuquerque, has provided insight into the mechanisms by which one of these changes impacts both CF and the complications of CF. These data provide support for ongoing clinical trials and raise the possibility of new targets for the development of drugs to treat individuals with CF.
CFTR mutations that cause CF make a compartment of the cell known as the TGN more acidic than it is in cells from healthy individuals. In this study, in vitro analysis revealed that hyperacidification of the TGN causes increased activity of a protein known as furin. Through increased production of a soluble factor known as TGF-beta this augmented the production of collagen (which in situ is associated with tissue fibrosis, a characteristic of damaged lung in individuals with CF) and suppressed the ability of human macrophages to kill the bacterium Pseudomonas aeruginosa, infection with which is a major complication for individuals with CF. This study provides strong support for the use of chloroquine (which counteracts high levels of TGN acidity) to treat CF, something that is currently being tested in clinical trials, and identifies furin inhibitors as potential new therapeutics for the treatment of CF.
Journal of Clinical Investigation
Cystic Fibrosis Current Events and Cystic Fibrosis News RSSResearchers describe how cells take out the trash to prevent disease
Garbage collectors are important for removing trash; without them waste accumulates and can quickly become a health hazard. Similarly, individual cells that make up such biological organisms as humans also have sophisticated methods for managing waste.
Children with cystic fibrosis not well covered by guidelines for vitamin D needs
Existing recommendations for treating vitamin D deficiency in children with cystic fibrosis (CF) are too low to cover the serious need, leaving most at high risk for bone loss and rickets, according to researchers at Johns Hopkins Children's Center.
New approach to treating cystic fibrosis lung infection shows promise
Researchers at the University of Calgary have found a new method of fighting severe lung infections in people with cystic fibrosis (CF). These findings are published in Proceedings of the National Academy of Science USA, this week.
Protein opens hope of treatment for cystic fibrosis patients
Scientists have finally identified a direct role for the missing protein that leaves cystic fibrosis patients open to attack from lung-damaging bacteria, the main reason most of them die before their 35th birthday, scientists heard today (Thursday 11 September 2008) at the Society for General Microbiology's Autumn meeting being held this week at Trinity College, Dublin.
Rattlesnake-type poisons used by superbug bacteria to beat our defenses
Colonies of hospital superbugs can make poisons similar to those found in rattlesnake venom to attack our bodies' natural defences, scientists heard today (Monday 8 September 2008) at the Society for General Microbiology's Autumn meeting being held this week at Trinity College, Dublin.
Clearing the airways in cystic fibrosis
By manipulating the machinery used by our cells for quality control, researchers from the University of Pittsburgh have found a way to restore the function of cystic fibrosis (CF) airway cells.
PTC124 shows activity in cystic fibrosis; Phase 2 proof-of-concept data published in Lancet
New phase 2 data published today in The Lancet show that the investigational oral drug PTC124 demonstrates activity in nonsense-mutation cystic fibrosis (CF). The data show that treatment with PTC124 results in statistically significant improvements in the chloride channel function of patients with nonsense-mutation CF. The study was conducted at the Hadassah Hebrew University Hospital in Jerusalem, Israel and sponsored by PTC Therapeutics (PTC).
Breaking the 'mucus barrier' with a new drug delivery system
Chemical engineers from Johns Hopkins University have broken the "mucus barrier," engineering the first drug-delivery particles capable of passing through human mucus - regarded by many as nearly impenetrable - and carrying medication that could treat a range of diseases. Those conditions include lung cancer, cervical cancer and cystic fibrosis, the research noted in a presentation scheduled for the 236th National Meeting of the American Chemical Society.
NIH scientists find a novel mechanism that controls the development of autoimmunity
Scientists at the National Institutes of Health (NIH) have found a mechanism in the immune systems of mice that can lead to the development of autoimmune disease when turned off.
Researchers discover cell's 'quality control' mechanism
Researchers in Japan and Canada have discovered a key component of the quality control mechanism that operates inside human cells - sometimes too well. The breakthrough has significant implications for the development of new treatments for cystic fibrosis (CF) and some other hereditary diseases, the researchers say. Their results were published July 25 in the journal Science.
More Cystic Fibrosis Current Events and Cystic Fibrosis News Articles
 | Cystic Fibrosis: Everything You Need To Know (Your Personal Health) by Wayne Kepron Cystic fibrosis afflicts approximately 30,000 Americans. The average age of survival has been steadily increasing, but not quickly enough: a child born with cystic fibrosis today can only expect to live 35 to 40 years. In this valuable new addition to the Your Personal Health series, Dr. Wayne Kepron offers a comprehensive look at the disease that afflicts so many young people. Topics... |
 | David's Promise: A Young Man's Struggle with Cystic Fibrosis by Kathy Sykes What would it be like to navigate this life under the shadow of a deadly disease? How would you cope, both physically and psychologically? Approximately 30,000 people in our country live with the devastating illness, cystic fibrosis, and one in twenty Caucasians are carriers of its gene. Many among us are affected by CF, but few really know much about it. David's Promise delivers an in-depth... |
 | Cystic Fibrosis by Margaret Hodson, Duncan Geddes, Andrew Bush This international and authoritative work, which brings together current knowledge in the field of cystic fibrosis, has become established in previous editions as a leading reference in the field. The third edition continues to provide everything that the clinician or allied health professional treating patients with cystic fibrosis will need in a single manageable volume. Thoroughly revised... |
 | With Every Breath: stories by and about people living with cystic fibrosis Over 30,000 people in the US have cystic fibrosis, a life-threatening genetic disease that affects the lungs and digestive system. Physically, fighting it is a very perilous struggle. Emotionally, coping can oftentimes be even harder. With Every Breath consists of stories by and about people living with cystic fibrosis. Created to motivate, inspire, and generate positivity for those living... |
 | Cystic Fibrosis (Genetic Diseases) by Maxine Rosaler |
 | Taking Cystic Fibrosis to School by Cynthia S. Henry, Cynthia S. Henry, Tom Dineen These beautifully illustrated and fun-to-read storybooks simplify and normalize complicated childhood conditions, like cystic fibrosis. When read aloud, other children can identify why a peer may be treated differently and begin to empathize with them. In addition, children whose conditions set them apart as being different begin to feel accepted and safe. Each book includes a Kids' Quiz to... |
 | The Power of Two: A Twin Triumph over Cystic Fibrosis by Isabel Stenzel Byrnes; Anabel Stenzel The tragedy of cystic fibrosis has been touchingly recounted before, but this is the first book to portray the symbiotic relationship between twins who share this life-threatening disease through adulthood. Isabel Stenzel Byrnes and Anabel Stenzel tell of their struggle to pursue normal lives while grappling with the realization that they might die young. Their story reflects the physical and... |
 | Cadberry's Letters by Jennifer Racek Cadberry's mom is very forgetful. Every time they visit his doctor together she talks about C & F. She never remembers the other letters. When Cadberry draws a set of letters to help his mother remember them all, he discovers what C & F really mean and how those two tiny letters affect so much of his life. Developed for pre-school-age children, Cadberry's Letters uses simple, easy-to-follow... |
 | Cystic Fibrosis: The Ultimate Teen Guide (It Happened to Me (the Ultimate Teen Guide)) by Melanie Ann Apel The day-to-day dealings of life as a cystic fibrosis patient are described through a series of flashbacks to a time when the author was an 18-year-old volunteer at cystic fibrosis overnight camp. From diagnosis to death, this book leaves no aspect of CF untold as it includes a description of the illness; a comprehensive discussion of who gets the disease and why; an explanation of the procedures... |
 | Cystic Fibrosis and You by Emilie Sasso This book is all about having a positive attitude in life and accomplishing your dreams. My hope in writing this book is to give encouragement to children and teens with cystic fibrosis. My sisters Bonnie and Kate accomplished everything in this book and more with CF. Never let cystic fibrosis hold you back from living life to the... |
| © 2008 BrightSurf.com |