Anno2010
AutoriMoran O
AbstractMutations of the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis, a hereditary lethal disease. CFTR is a chloride channel expressed in the apical membrane of epithelia. It is activated by cAMP dependent phosphorylation and gated by the binding of ATP. The impaired chloride transport of some types of cystic fibrosis mutations could be pharmacologically solved by the use of chemical compounds called potentiators. Here it is undertaken the construction of a model of the CFTR activation pathways, and the possible modification produced by a potentiator application. The model yields a novel mechanism for the potentiator action, describing the activatory and inhibitory activities on two different positions in the CFTR activation pathway.
RivistaJournal Of Theoretical Biology
ISSN
Impact factor
Volume262
Pagina inizio73
Pagina fine79
Autori IBFOscar MORAN
Linee di Ricerca IBFMD.P01.009.001
Sedi IBFIBF.GE