Open AccessAirway acidification initiates host defense abnormalities in cystic fibrosis mice
Author(s) -
Viral S. Shah,
David K. Meyerholz,
Xiao Xiao Tang,
Leah R. Reznikov,
Mahmoud H. Abou Alaiwa,
Sarah E. Ernst,
Philip H. Karp,
Christine Wohlford-Lenane,
Kristopher P. Heilmann,
Mariah Leidinger,
Patrick D. Allen,
Joseph Zabner,
Paul B. McCray,
Lynda S. Ostedgaard,
David A. Stoltz,
Christoph O. Randak,
Michael J. Welsh
Publication year - 2016
Publication title -
science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 12.556
H-Index - 1186
eISSN - 1095-9203
pISSN - 0036-8075
DOI - 10.1126/science.aad5589
Subject(s) - cystic fibrosis , cystic fibrosis transmembrane conductance regulator , airway , immunology , biology , regulator , host (biology) , medicine , genetics , gene , surgery
Airway infections put to an acid test Most people with cystic fibrosis suffer from chronic respiratory infections. The mechanistic link between this symptom and the genetic cause of the disease (mutations that compromise the function of the cystic fibrosis transmembrane conductance regulator, CFTR) is not fully understood. Studying animal models, Shahet al. find that in the absence of functional CFTR, the surface liquid in the airways becomes acidic, which impairs host defenses against infection. This acidification occurs through the action of a proton pump called ATP12A. Molecules inhibiting ATP12A could potentially be developed into useful drugs.Science , this issue p.503
Accelerating Research
Robert Robinson Avenue,
Oxford Science Park, Oxford
OX4 4GP, United Kingdom
Address
John Eccles HouseRobert Robinson Avenue,
Oxford Science Park, Oxford
OX4 4GP, United Kingdom