Ontology highlight
ABSTRACT:
SUBMITTER: Shah VS
PROVIDER: S-EPMC4852973 | biostudies-literature | 2016 Jan
REPOSITORIES: biostudies-literature
Shah Viral S VS Meyerholz David K DK Tang Xiao Xiao XX Reznikov Leah L Abou Alaiwa Mahmoud M Ernst Sarah E SE Karp Philip H PH Wohlford-Lenane Christine L CL Heilmann Kristopher P KP Leidinger Mariah R MR Allen Patrick D PD Zabner Joseph J McCray Paul B PB Ostedgaard Lynda S LS Stoltz David A DA Randak Christoph O CO Welsh Michael J MJ
Science (New York, N.Y.) 20160101 6272
Cystic fibrosis (CF) is caused by mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel. In humans and pigs, the loss of CFTR impairs respiratory host defenses, causing airway infection. But CF mice are spared. We found that in all three species, CFTR secreted bicarbonate into airway surface liquid. In humans and pigs lacking CFTR, unchecked H(+) secretion by the nongastric H(+)/K(+) adenosine triphosphatase (ATP12A) acidified airway surf ...[more]