OBSERVATION'S |
NMR study of genetically modified form of CFTR that causes cystic fibrosis when inherited reveals new structural informa tion that could help researchers develop cystic fibrosis therapeutics. The study focused on a key region of CFTR from which most cases of cystic fibrosis arise. They find that the region has a tendency to form an alpha-helix & that the helix is destabilized in the mutated version of CFTR, in which aminoacid residue phenylalanine-508 is missing. The study suggests that the mutant CFTR gets trapped in the endoplas mic reticulum of patients airway cells because of its lower helical-forming potential, & that this prevents it from functio ning properly. The researchers believe compds could be screened for their ability to correct the CFTR misfolding defect, & that agents so identified could be considered for clinical trials |
UPDATE | 05.99 |
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LITERATURE REF. | This data is not available for free |
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