FRANCIS LEROY & OLIVIER WAUTIER, BIOCOSMOS / SCIENCE PHOTO LIBRARY FRANCIS LEROY & OLIVIER WAUTIER, BIOCOSMOS / SCIENCE PHOTO LIBRARY
Cystic fibrosis ion channel. Animation of the cystic fibrosis transmembrane conductance regulator. This is a chloride ion channel found in the cell membranes of epithelial cells. Mutations in the gene that code for this protein cause cystic fibrosis. The mutations disrupt fluid flow and result in the production of abnormally thick mucus in the lungs and pancreas. The mucus in the lungs encourages bacterial growth, leading to severe respiratory infections. The mucus in the pancreas blocks the release of digestive enzymes, resulting in malabsorption. When functioning normally the channel is activated by the phosphorylation of its regulatory 'R' domain (green) and the hydolysis of ATP (adenosine triphospahte, blue) bound to its nucleotide binding domains (orange). Activation releases chloride ions (small dots) to the exterior of the cell.
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