Navigation Links
Pig model of cystic fibrosis improves understanding of disease
Date:3/16/2011

R gene," said David Stoltz, M.D., Ph.D., UI assistant professor of internal medicine and senior study author. "For example, there already are drugs known as "correctors" being tested. These drugs help CFTR move from inside the cell to its correct location on the cell surface.

"The pig model could help us develop and test more corrector drugs, and it will also help us better understand why the protein is misprocessed in the first place," Stoltz added. "If we understand what is going wrong, we may be able to develop new therapies that can target the problem and allow more of the CFTR to make it to the cell surface, which may alleviate the disease symptoms."

In 2008, the UI team and colleagues at University of Missouri created pigs that were missing the CFTR protein. These animals developed CF disease symptoms that closely mimicked the human disease. In the new pig model, the animals have two copies of the CFTR gene containing the most common CF-causing mutation, which is known as the delta F508 mutation. These pigs also develop CF symptoms similar to the human disease. In particular, the CF pigs are born with gastrointestinal disease and develop lung disease over time.

By studying the protein in the pigs, the researchers were able to show that most of the CFTR protein is misprocessed and gets degraded, but a small amount of the protein does get to the cell membrane where it is able to form active chloride channels. However, the level of activity is only about 6 percent of the activity found in normal pigs with fully functional CFTR channels. The study shows that this small amount of CFTR activity is not sufficient to prevent CF disease in the pigs.

CF is a recessive disease, meaning a person with one mutated copy and one good copy of the CFTR gene is a "carrier" but does not have CF. This suggests that 50 percent of normal CFTR activity is sufficient for health. The question has always been, 'Is there a minimal amount of active CFTR
'/>"/>

Contact: Jennifer Brown
jennifer-l-brown@uiowa.edu
319-356-7124
University of Iowa Health Care
Source:Eurekalert  

Page: 1 2 3

Related biology news :

1. UCSF report describes new model for neurodegeneration
2. New model shows importance of feet, toes in body balance
3. Sink or source? A new model to measure organic carbon in surface waters
4. UofL biostatistician to develop statistical model that could help answer medical questions
5. DOE grant joins climate modeling with local, regional empirical data
6. Study shows rapamycin reverses myocardial defects in mouse model of LEOPARD syndrome
7. Modell Chair in Pediatric Immunology established
8. New model reveals pesticide-free method that takes a bite out of mosquito-borne disease
9. New discoveries improve climate models
10. Mathematical model could help predict and prevent future extinctions
11. Purdue team creates engineered organ model for breast cancer research
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Pig model of cystic fibrosis improves understanding of disease
(Date:7/9/2014)... Bacterial infections usually announce themselves with pain and fever but ... are those that are sneaky and hard to beat. Now, ... the form of tiny DNA pyramids. Published in the journal ... the nanopyramids can flag bacteria and kill more of them ... note that some infectious pathogens can lie in wait, undetectable ...
(Date:7/9/2014)... the amphibians with the highest distribution in the Iberian ... where it comes into contact with the red swamp ... by the Spaniard Germn Orizaola from the University of ... have developed a defensive response to the invasive species. ... they co-exist with the crayfish. , Numerous invasive organisms ...
(Date:7/9/2014)... N-methyl-D-aspartate receptor antagonists can alleviate the symptoms ... irreversible cognitive dysfunction and effectively scavenge amyloid ... peptide (Aβ) vaccines reduced and eliminated Aβ ... mouse model, and significantly improved behavioral and ... team, First Affiliated Hospital of China Medical ...
Breaking Biology News(10 mins):Frogs have developed rapid defences against the red swamp crayfish 2Frogs have developed rapid defences against the red swamp crayfish 3
... 2011) Proving that technological entrepreneurship can have ... fields, renowned innovator, materials scientist and applied physicist ... recipient of the 2011 $500,000 Lemelson-MIT Prize. Rogers, ... products integral to human health, fiber optics, semiconductor ...
... A new study provides support for Darwin,s hypothesis ... more closely related species than those distantly related. ... study contains the strongest direct experimental evidence yet ... species extinction occurred more frequently and more rapidly ...
... rapidly spreading amphibian disease, has reached a site near ... the entire mountainous neotropics to be free of the ... Rescue and Conservation Project, a consortium of nine U.S. ... of frogs in imminent danger of extinction. ...
Cached Biology News:Materials scientist and entrepreneur Dr. John Rogers awarded $500,000 Lemelson-MIT Prize 2Materials scientist and entrepreneur Dr. John Rogers awarded $500,000 Lemelson-MIT Prize 3Materials scientist and entrepreneur Dr. John Rogers awarded $500,000 Lemelson-MIT Prize 4New study supports Darwin's hypothesis on competition between species 2New study supports Darwin's hypothesis on competition between species 3Scientists find deadly amphibian disease in the last disease-free region of central America 2Scientists find deadly amphibian disease in the last disease-free region of central America 3
(Date:1/15/2014)... The Microcompetition with Foreign DNA theory explains how latent viruses ... is the Epstein Barr Virus (EBV), and one of these ... chronic inflammatory disease that destroys the body’s joints. As predicted ... high concentrations of EBV DNA in their bodies (1). ...
(Date:1/15/2014)... Jan. 15, 2014 TaiGen Biotechnology Company, Limited ("TaiGen") ... with R-Pharm, a leading Russian pharmaceutical company, to develop ... Russian Federation , Turkey ... (CIS). Nemonoxacin is a novel antibiotic for the treatment of ...
(Date:1/14/2014)... Calif. , Jan. 14, 2014 As pet owners drew ... doubt many of them wrote: "Take better care of my furry ... even designer clothes and top-brand carrying cases to take the little ... parents might also feel compelled to buy some pricey toys at ...
(Date:1/14/2014)... January 14, 2014 In recent years, ... and methods in product development and promotion has led ... This mistrust, fueled by concerns about the insidious impact ... reports of spectacular fines to the world’s biggest pharmas ...
Breaking Biology Technology:Study: Rheumatoid Arthritis (RA) Patients Have EBV; The CBCD Says this is Consistent with Microcompetition 2Study: Rheumatoid Arthritis (RA) Patients Have EBV; The CBCD Says this is Consistent with Microcompetition 3TaiGen Biotechnology Signed Exclusive License Agreement with R-Pharm for Nemonoxacin (Taigexyn(R)) 2TaiGen Biotechnology Signed Exclusive License Agreement with R-Pharm for Nemonoxacin (Taigexyn(R)) 3TaiGen Biotechnology Signed Exclusive License Agreement with R-Pharm for Nemonoxacin (Taigexyn(R)) 4New Year's Resolution: Give Pets the Gift of Top-Notch Health Care 2The Sunshine Act: Necessary Regulation or Unnecessary Dysregulation? New Life Science Webinar Hosted by Xtalks and IRB Services 2The Sunshine Act: Necessary Regulation or Unnecessary Dysregulation? New Life Science Webinar Hosted by Xtalks and IRB Services 3
... ... volumes of research records. , ... Reston, VA (PRWEB) January 28, 2010 -- Surety, LLC , the ... drug discovery and development company, has selected Surety,s AbsoluteProof® trusted, digital timestamping service for ...
... ... , , ... Custom Data, the underlying source of Scopus.com, the world,s,largest abstract and citation ... analysis between scientific,articles and patents. JST, as the funding agency for promoting ...
... , , , , , , , , ... Biotech Ltd. ( SVA),("Sinovac" or the "Company"), a leading China ... of 10,000,000 common,shares at $5.75 per common share. ... to an additional 1,500,000 common,shares on the same terms and conditions. ...
Cached Biology Technology:Biotech Company Ansaris Selects Surety for Integrity and Authenticity Protection of Its Lab Informatics Data 2Biotech Company Ansaris Selects Surety for Integrity and Authenticity Protection of Its Lab Informatics Data 3Biotech Company Ansaris Selects Surety for Integrity and Authenticity Protection of Its Lab Informatics Data 4Japan Science and Technology Agency (JST) Selects Scopus Custom Data for Citation Linkage Analysis Between Scientific Articles and Patents 2Japan Science and Technology Agency (JST) Selects Scopus Custom Data for Citation Linkage Analysis Between Scientific Articles and Patents 3Japan Science and Technology Agency (JST) Selects Scopus Custom Data for Citation Linkage Analysis Between Scientific Articles and Patents 4Japan Science and Technology Agency (JST) Selects Scopus Custom Data for Citation Linkage Analysis Between Scientific Articles and Patents 5Japan Science and Technology Agency (JST) Selects Scopus Custom Data for Citation Linkage Analysis Between Scientific Articles and Patents 6Sinovac Announces Upsizing and Pricing of Common Share Offering 2Sinovac Announces Upsizing and Pricing of Common Share Offering 3
Collected from 8-12 week-old New Zealand or Californian rabbits.,Supplied in lyophilized form....
Antibody specific for phosphorylattion of histone H3 that occurs during mitosis. Rabbit polyclonal serum. Antigen: Synthetic peptides derived from histone H3, phosphorylated at serines 10 or 28...
5 minutes from gel band to purified DNA...
...
Biology Products: