Protein structure could unlock new treatments for cystic fibrosis

(University of Zurich) Biochemists at the University of Zurich have used cryo-electron microscopy to determine the detailed architecture of the chloride channel TMEM16A. This protein is a promising target for the development of effective drugs to treat cystic fibrosis.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news

Related Links:

A new device — wearable, wireless and battery free — improves the ability to monitor and diagnose health problems by analyzing the sweat on your skin.
Source: NYT Health - Category: Consumer Health News Authors: Tags: Sweating Sensors Cystic Fibrosis Wearable Computing Data-Mining and Database Marketing Exercise Skin Diabetes Source Type: news
Regulated mucin secretion is essential for the formation of the mucus layer that protects the underlying epithelial cells from foreign particles. Alterations in the quantity or quality of secreted mucins are therefore detrimental to airway and colon physiology. Based on various biochemical assays in several human cell lines, we report here that Na+/Ca2+ exchanger 2 (NCX2) works in conjunction with transient receptor potential cation channel subfamily M member 4 (TRPM4), and perhaps TRPM5, Na+ channels to control Ca2+-mediated secretion of both mucin 2 (MUC2) and MUC5AC from HT29-18N2 colonic cancer cells. Differentiated no...
Source: Journal of Biological Chemistry - Category: Chemistry Authors: Tags: Cell Biology Source Type: research
Source: Infectious Diseases - Category: Infectious Diseases Authors: Source Type: research
Publication date: Available online 16 January 2019Source: The Lancet Respiratory MedicineAuthor(s): Emma Grainger
Source: The Lancet Respiratory Medicine - Category: Respiratory Medicine Source Type: research
Abstract The gastrointestinal phenotype of cystic fibrosis (CF) features intestinal bile acid (BA) malabsorption, impaired intestinal farnesoid X receptor (FXR) activation and consequently reduced fibroblast growth factor 19 (FGF19, FGF15 in mice) production. The osmotic laxative polyethylene glycol (PEG) has been shown to decrease intestinal mucus accumulation in CF mice and could, by doing so, improve BA reabsorption. Here we determined the effect of PEG on BA excretion and FXR-FGF15 signaling in CF mice. Male Cftr-/-tm1Unc (CF) and wild type (WT) littermates were administered PEG 4000 in drinking water and fed ...
Source: Am J Physiol Gastroi... - Category: Gastroenterology Authors: Tags: Am J Physiol Gastrointest Liver Physiol Source Type: research
A. Crespo Wearable potentiometric sensors have received considerable attention owing to their great potential in a wide range of physiological and clinical applications, particularly involving ion detection in sweat. Despite the significant progress in the manner that potentiometric sensors are integrated in wearable devices, in terms of materials and fabrication approaches, there is yet plenty of room for improvement in the strategy adopted for the sample collection. Essentially, this involves a fluidic sampling cell for continuous sweat analysis during sport performance or sweat accumulation via iontophoresis induct...
Source: Sensors - Category: Biotechnology Authors: Tags: Review Source Type: research
The epidemiology and natural history of cystic fibrosis (CF) is changing. Predicted median survival has increased over the past five decades, from less than 12 to more than 45 years in most Western countries, along with an estimated increase of the number of patients: by 20% in children and by 75% in adults, over the period 2010 to 2025 [1]. The number of adults (age ≥18 years) with CF is already larger than the number of children [2, 3]. This is a consequence of improvements in drug therapies and in the management of patients delivered by multidisciplinary teams in specialised CF centres, resulting in prolong...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Original Articles: Correspondence Source Type: research
Publication date: Available online 15 January 2019Source: The Lancet Respiratory MedicineAuthor(s): Charles S Haworth, Diana Bilton, James D Chalmers, Angela M Davis, Juergen Froehlich, Igor Gonda, Bruce Thompson, Adam Wanner, Anne E O'DonnellSummaryBackgroundIn patients with non-cystic fibrosis bronchiectasis, lung infection with Pseudomonas aeruginosa is associated with frequent pulmonary exacerbations and admission to hospital for treatment, reduced quality of life, and increased mortality. Although inhaled antibiotics are conditionally recommended for long-term management of non-cystic fibrosis bronchiectasis with freq...
Source: The Lancet Respiratory Medicine - Category: Respiratory Medicine Source Type: research
Conclusion:Current limitations of UTE MRI include long scan times, poor delineation of thin-walled structures (e.g. cysts and reticulation) due to limited spatial resolution, low signal to noise ratio, and imperfect motion compensation. Despite these limitations, UTE MRI can now be considered as an alternative to multidetector computed tomography for the longitudinal follow-up of the morphological changes from lung diseases in neonates, children, and young adults, particularly as a complement to the unique functional capabilities of MRI.
Source: Academic Radiology - Category: Radiology Source Type: research
Conclusions: The present study demonstrated that TLR4 and PPARα receptors would mediate the earliest control of bacterial replication as well as proinflammatory responses to PA infections, and in particular that PPARα receptors are needed to prevent an excessive inflammatory response, as in the control of the inflammasome complex NLP3 activation.
Source: Shock - Category: Emergency Medicine Tags: Basic Science Aspects Source Type: research
More News: Biochemistry | Chloride | Cystic Fibrosis