CFTR (cystic fibrosis transmembrane conductance regulator) protein expression in well-differentiated human bronchial epithelial cultures. Well-differentiated cultures derived from human bronchial epithelial tissues were immunostained with CFTR and tubulin antibodies and analyzed on a Leica SP2 laser confocal microscope. The image represents an overlay of the DIC (grayscale), CFTR (red), cilia (tubulin, green), and nuclei (DAPI, blue) confocal planes, and depicts an epithelial cell sheet that contains a group of ciliated cells surrounding a goblet cell (bottle-shaped cell with no cilia). CFTR is expressed only at the apical membrane of ciliated cells, but not goblet cells. Magnification x190. Reproduced from Kreda et al 2005 Miol Biol Cell 16, 2154 with permission of ASCB / MBC.
The CFTR protein functions as a channel across the membrane of cells that produce mucus, sweat, saliva, tears, and digestive enzymes. The channel transports chloride ions into and out of cells. The transport of chloride ions helps control the movement of water in tissues, which is necessary for the production of thin, freely flowing mucus. Mucus is a slippery substance that lubricates and protects the lining of the airways, digestive system, reproductive system, and other organs and tissues.
More than 1,000 mutations in the CFTR gene have been identified in people with cystic fibrosis. The resulting abnormal channel breaks down shortly after it is made, so it never reaches the cell membrane to transport chloride ions and the movement of water into and out of cells.
As a result, cells that line the passageways of the lungs, pancreas, and other organs produce mucus that is abnormally thick and sticky. The abnormal mucus obstructs the airways and glands, leading to the characteristic signs and symptoms of cystic fibrosis.
Next up in my “all OCs deserve a mystic arte cut-in” project is Miol. I think of all my OCs he might be my favourite to draw, which you would never know from how few pictures I have of him… but every sketch seems to turn into a picture I adore. <3 I accidentally drew him with his secondary weapon here, I’d finished the sketch before I noticed and I really liked the pose so I kept it. For the record his main weapon is a bow.