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Titolo:
Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid
Autore:
Bals, R; Weiner, DJ; Meegalla, RL; Accurso, F; Wilson, JM;
Indirizzi:
Wistar Inst Anat & Biol, Philadelphia, PA 19104 USA Wistar Inst Anat & Biol Philadelphia PA USA 19104 ladelphia, PA 19104 USA Univ Penn, Dept Med, Inst Human Gene Therapy, Philadelphia, PA 19104 USA Univ Penn Philadelphia PA USA 19104 e Therapy, Philadelphia, PA 19104 USA Univ Penn, Dept Mol & Cellular Engn, Inst Human Gene Therapy, Philadelphia, PA 19104 USA Univ Penn Philadelphia PA USA 19104 e Therapy, Philadelphia, PA 19104 USA Childrens Hosp Philadelphia, Div Pulm Med, Philadelphia, PA USA Childrens Hosp Philadelphia Philadelphia PA USA ed, Philadelphia, PA USA Univ Colorado, Sch Med, Denver, CO USA Univ Colorado Denver CO USAUniv Colorado, Sch Med, Denver, CO USA Childrens Hosp, Denver, CO 80218 USA Childrens Hosp Denver CO USA 80218Childrens Hosp, Denver, CO 80218 USA
Titolo Testata:
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
fascicolo: 1, volume: 25, anno: 2001,
pagine: 21 - 25
SICI:
1044-1549(200107)25:1<21:SAOAAI>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
HUMAN BRONCHIAL EPITHELIA; HUMAN LUNG; HUMAN BETA-DEFENSIN-1; GENE-TRANSFER; PEPTIDE; XENOGRAFTS; DISEASE; INFANTS; CFTR;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
17
Recensione:
Indirizzi per estratti:
Indirizzo: Wilson, JM Wistar Inst Anat & Biol, 3601 Spruce St 204, Philadelphia, PA 19104 USA Wistar Inst Anat & Biol 3601 Spruce St 204 Philadelphia PA USA 19104
Citazione:
R. Bals et al., "Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid", AM J RESP C, 25(1), 2001, pp. 21-25

Abstract

The link between the genetic defect in cystic fibrosis (CF) and the recently described breach in pulmonary host defense has focused on the role of salt and water metabolism in the airways. Using a human bronchial xenograft model we demonstrate a salt-independent abnormality in bacterial killing in CF airway surface fluid (ASF). Biochemical characterization implicates the absence or dysfunction of a molecule critical to the constitution of normalbacterial killing. Our study suggests that CF transmembrane conductance regulator (CFTR) deficiency causes a primary abnormality in the composition of ASF that leads to a salt-independent defect in host defense. Importantly,this defect is corrected by adenovirus-mediated gene transfer of CFTR.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 16:45:35