CFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis
نویسندگان
چکیده
منابع مشابه
Strategies to circumvent the CFTR defect in cystic fibrosis
Mutations within the gene encoding for the chloride ion channel CFTR results in cystic fibrosis, the most common autosomal recessive genetic disease in the Caucasian population. CFTR regulates absorption and secretion mechanisms across intestinal and airway mucosae. Although the intestinal phenotype can be clinically handled, chronic infection and inflammation of the lungs of CF patients remain...
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Chronic pancreatitis (CP) is a progressive and irreversible inflammatory and fibrotic disease with no cure. Unlike acute pancreatitis (AP), we find that alternatively activated macrophages (AAMs) are dominant in mouse and human CP. AAMs are dependent on interleukin (IL)-4 and IL-13 signalling, and we show that mice lacking IL-4Rα, myeloid-specific IL-4Rα and IL-4/IL-13 were less susceptible to ...
متن کاملNovel CFTR Mutations in Two Iranian Families with Severe Cystic Fibrosis
Background: Cystic fibrosis (CF) is a common autosomal recessive disorder that affects many body systems and is produced by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CF is also the most frequently inherited disorder in the West. The aim of this study was to detect the mutations in the CFTR gene in two Iranian families with CF. Methods: After DNA extractio...
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Macrophages are innate immune cells that play an important role in activation of the immune response and wound healing. Pathogens that require T helper-type 2 (Th2) responses for effective clearance, such as parasitic worms, are strong inducers of alternatively activated or M2 macrophages. However, infections such as bacteria and viruses that require Th1-type responses may induce M2 as a strate...
متن کاملCFTR and Ca2+ Signaling in Cystic Fibrosis
Among the diverse physiological functions exerted by calcium signaling in living cells, its role in the regulation of protein biogenesis and trafficking remains incompletely understood. In cystic fibrosis (CF) disease the most common CF transmembrane conductance regulator (CFTR) mutation, F508del-CFTR generates a misprocessed protein that is abnormally retained in the endoplasmic reticulum (ER)...
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ژورنال
عنوان ژورنال: Journal of Cystic Fibrosis
سال: 2017
ISSN: 1569-1993
DOI: 10.1016/j.jcf.2017.03.011