 |
|
Cystic Fibrosis Proteins Photographed Interacting
December 10, 2007BIRMINGHAM, Ala. - New microscopic pictures show the first-ever physical evidence of interaction between two proteins involved in Cystic Fibrosis (CF) disease.
Researchers at the University of Alabama at Birmingham (UAB) studied a CF-causing protein and another protein called epithelial sodium channel, or ENaC. Both proteins are known to play a role in CF, but exactly what role each plays is unclear.
The new pictures provide physical proof that these two proteins occur close enough to interact with each other, the UAB researchers said. When that interaction fails then a dangerous biochemical imbalance occurs in people diagnosed with CF.
Knowing more about this protein proximity will help scientists better understand the biological underpinnings of CF and may speed the discovery of new drugs to treat or cure the disease. CF affects 70,000 people worldwide, 30,000 in the United States.
The findings are published in the Journal of Biological Chemistry, which included a color photo from the UAB study on its cover.
"Our findings suggest a direct interaction between these two proteins and their proximity makes the evidence convincing," said Bakhrom Berdiev, M.D., Ph.D., a UAB assistant professor of cell biology and lead author on the study.
The study was conducted using a laser-scanning microscope that captured images of the two proteins "within reach of each other. The absence of this interaction could shape the development of CF," Berdiev said.
Normally, both proteins help make up the lining of the lungs and other membranes, and keep the balance of water and salt at safe levels. In the case of CF, ENaC is allowed to hyper-function in the presence of the CF-causing protein and disturb the balance of water and salt.
When this happens, a sticky mucus usually forms inside the airways which can lead to chronic lung infections, the most dangerous symptom of CF. Other symptoms involve digestive problems.
With the discovery of the CF gene in 1989, many scientists began searching for ways to repair the DNA or repair the faulty protein encoded by the DNA.
The new Journal of Biological Chemistry study shows that future research should look at other molecules not directly linked to CF but are involved in the disease process and its side effects, Berdiev said.
Also, the new pictures add to an ever-changing biophysical "map" of CF used to design future research.
The new pictures were done through collaboration between UAB's departments of Cell Biology, Physiology & Biophysics, High Resolution Imaging Facility and the Gregory Fleming James Cystic Fibrosis Research Center at UAB. Other collaborators are from Ohio State University, the University of Toronto, Ontario, the National Heart Lung and Blood Institute in Bethesda, Maryland and Lambert Instruments in the Netherlands.
Funding for the study came from the National Institutes of Health and the Cystic Fibrosis Foundation.
University of Alabama at Birmingham
Knowing more about this protein proximity will help scientists better understand the biological underpinnings of CF and may speed the discovery of new drugs to treat or cure the disease. CF affects 70,000 people worldwide, 30,000 in the United States.
The findings are published in the Journal of Biological Chemistry, which included a color photo from the UAB study on its cover.
"Our findings suggest a direct interaction between these two proteins and their proximity makes the evidence convincing," said Bakhrom Berdiev, M.D., Ph.D., a UAB assistant professor of cell biology and lead author on the study.
The study was conducted using a laser-scanning microscope that captured images of the two proteins "within reach of each other. The absence of this interaction could shape the development of CF," Berdiev said.
Normally, both proteins help make up the lining of the lungs and other membranes, and keep the balance of water and salt at safe levels. In the case of CF, ENaC is allowed to hyper-function in the presence of the CF-causing protein and disturb the balance of water and salt.
When this happens, a sticky mucus usually forms inside the airways which can lead to chronic lung infections, the most dangerous symptom of CF. Other symptoms involve digestive problems.
With the discovery of the CF gene in 1989, many scientists began searching for ways to repair the DNA or repair the faulty protein encoded by the DNA.
The new Journal of Biological Chemistry study shows that future research should look at other molecules not directly linked to CF but are involved in the disease process and its side effects, Berdiev said.
Also, the new pictures add to an ever-changing biophysical "map" of CF used to design future research.
The new pictures were done through collaboration between UAB's departments of Cell Biology, Physiology & Biophysics, High Resolution Imaging Facility and the Gregory Fleming James Cystic Fibrosis Research Center at UAB. Other collaborators are from Ohio State University, the University of Toronto, Ontario, the National Heart Lung and Blood Institute in Bethesda, Maryland and Lambert Instruments in the Netherlands.
Funding for the study came from the National Institutes of Health and the Cystic Fibrosis Foundation.
University of Alabama at Birmingham
Cystic Fibrosis Current Events and Cystic Fibrosis News RSSCausative gene of a rare disorder discovered by sequencing only protein-coding regions of genome
For the first time, scientists have successfully used a method called exome sequencing to quickly discover a previously unknown gene responsible for a mendelian disorder.
Bacteria 'launch a shield' to resist attack
Researchers from the University of Copenhagen and the Technical University of Denmark along with other collaborators in Denmark and the US found that the bacterium Pseudomonas aeruginosa can 'switch on' production of molecules that kill white blood cells - preventing the bacteria being eliminated by the body's immune system.
Protein critical for insulin secretion may be contributor to diabetes
A cellular protein from a family involved in several human diseases is crucial for the proper production and release of insulin, new research has found, suggesting that the protein might play a role in diabetes.
New clinical guidelines for exacerbations in cystic fibrosis
The American Thoracic Society has released new clinical guidelines for the treatment of exacerbations in cystic fibrosis based on a review of the literature on current clinical practices.
Nanoparticle treatment for burns curbs infection, reduces inflammation
Treating second-degree burns with a nanoemulsion lotion sharply curbs bacterial growth and reduces inflammation that otherwise can jeopardize recovery, University of Michigan scientists have shown in initial laboratory studies.
Research Matters at the Arizona Health Sciences Center (AHSC): UA Researchers Seek Safer Cystic Fibrosis Test
Researchers from The University of Arizona Colleges of Pharmacy and Medicine are teaming up to try to invent a novel non-invasive lung test for cystic fibrosis sufferers.
Species diversity helps ASU researchers refine analyses of human gene mutations
In the new era of personalized medicine, physicians hope to provide earlier diagnoses and improve therapy by evaluating patients' genetic blueprints. But, as a new bioinformatics study emphasizes, the first step must be to correctly decipher the deluge of information locked in our DNA and determine its impact on human health.
New developments in reproductive medicine
Three out of ten women who undergo polar body diagnosis go on to have a child.
Faster, cheaper way to find disease genes in human genome passes initial test
University of Washington (UW) researchers have successfully developed a novel genome-analysis strategy for more rapid, lower cost discovery of possible gene-disease links.
Mutation responsible for cystic fibrosis also involved in muscle atrophy
Patients with cystic fibrosis (CF) usually experience significant muscle loss, a symptom traditionally considered to be a secondary complication of the devastating genetic disease.
More Cystic Fibrosis Current Events and Cystic Fibrosis News Articles
 |
Cystic Fibrosis: A Guide for Patient and Family by David M Orenstein (Editor) Univ. of Pittsburgh, PA. Text is designed specifically for patients with cystic fibrosis and their families. Explains the disease process, outlines the fundamentals of diagnosing and screening, and addresses the challenges of treatment for those living with CF. Includes new material on carrier testing, infection control, and more. Previous edition: c1997. Softcover. |
 |
Cystic Fibrosis: Everything You Need To Know (Your Personal Health) by Wayne Kepron MD FRCPC (Author) 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 include: - Symptoms of cystic fibrosis - Making a diagnosis - Complications of the disease - Treatments (including lung transplants) - Techniques and precautions in lung care - Transition into adulthood - Prospects for gene therapy - End-of-life issues Using diagrams, charts and case studies, Cystic Fibrosis is designed for patients, their families, and caregivers. It is both a quick... |
 |
With Every Breath: stories by and about people living with cystic fibrosis by Katherine Russell and Margot Russell (Author), Katherine Russell and Margot Russell (Editor) 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 with cystic fibrosis, this book is something you can open time and time again. Designed for all ages, this collection of diverse stories offers unique perspectives from patients, a CF doctor and nurse, and family members of those living with the illness. Patients aren't the only ones who will want to read With Every Breath as family members, friends, and doctors can all find inspiration when they... |
 |
Cystic Fibrosis (Facts) by Ann Harris (Author), Anne Thomson (Author) Cystic Fibrosis: The Facts provides a much needed simple and understandable source book about this disease. It is aimed at those living with Cystic Fibrosis (CF), either themselves or members of their families or their friends. The book explains clearly what is happening to the body in CF, what causes it and what treatment options are available for the different aspects of the disease. There are more detailed chapters for those wanting to find out about the genetics of the disease and specific aspects such as how it affects life choices and employment. It looks to the future in terms of potential new therapies for CF and provides useful information on organizations that can provide help and further information across those areas of the world where the disease is prevalent. |
 |
The Power of Two: A Twin Triumph over Cystic Fibrosis by Isabel Stenzel Byrnes; Anabel Stenzel (Author) 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 emotional challenges of a particularly aggressive form of CF and tells how the twins bicultural heritage Japanese and German influenced the way they coped. The Power of Two is an honest and gripping portrayal of day-to-day health care, the impact of chronic illness on marriage and family, and the importance of a support network to continuing survival. These two remarkable sisters have much to teach... |
 |
The Stones Applaud: How Cystic Fibrosis Shaped My Childhood by Teresa Anne Mullin (Author) Teresa Mullin was diagnosed with cystic fibrosis at four years of age, but it was not until she was nine that she learned most children with the disease were not expected to live to adulthood. What had been a nuisance soon became a force that molded her childhood, youth, and future. In The Stones Applaud, Mullin writes of absences from school, serving as a poster child, frequent hospitalizations, medical treatments, and most painful the isolation that came with cystic fibrosis, an inherited condition that damages the lungs and affects the digestive system. With dry humor and sharp insights, Mullin describes her battles with the disease, teachers, fellow students, and even medical professionals who tried to hold her back from experiencing life. Alternately funny, frank, poignant, and... |
 |
Cadberry's Letters by Jennifer Racek (Author) 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 language to explain Cystic Fibrosis and the daily care that goes along with it in terms young children can understand. Children will learn along with Cadberry about things like Pancreatic Enzymes, Chest Physical Therapy and more. Bright, full-page illustrations bring the story to life and feature a lot of the equipment and medications CF patients may use in their daily lives. |
 |
Cystic Fibrosis by Margaret Hodson (Author), Duncan Geddes (Author), Andrew Bush (Author) 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 and updated throughout, it reflects the significant advances that have been made in the field since the second edition published in 2000. Cystic Fibrosis outlines in detail the basic science that underlies the disease and its progression, putting it into a clinical context. Diagnostic and clinical aspects are covered in depth, as are monitoring the condition and the importance of... |
 |
Understanding Cystic Fibrosis (Understanding Health and Sickness Series) by Ph.D., Karen Hopkin (Author) Since the discovery of the CF gene in 1989, scientists have learned a great deal about the biology of this disease which strikes one child in every 3,300 births. With the gene pinpointed, scientists are now working on ways to replace it and are developing better tests for earlier diagnosis. Understanding CF charts the progress that has been made in a disease whose symptoms can range from mild respiratory distress to life-threatening lung infections |
 |
Taking Cystic Fibrosis to School by Cynthia S. Henry (Author), Cynthia S. Henry (Author), Tom Dineen (Author), Tom Dineen (Illustrator) 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 reinforce new information and Ten Tips for Teachers to provide additional facts and ideas for teacher use. In Taking Cystic Fibrosis to School, Jessie explains to her classmates that even though she has cystic fibrosis, she can still attend school. |
| © 2009 BrightSurf.com |