CareLit Fachartikel

Rescue of function in a cystic fibrosis mouse model by transfer of hematopoetic stem cells

A Munder, R Struß, A Schambach, B Tümmler · Pneumologie · 2014 · Heft 06

Dokument
1113518
CareLit-ID
Jahr
2014
Publikation
PDF
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Metadaten
DOI
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Bibliografische Angaben

Zeitschrift
Pneumologie
Autor:innen
A Munder, R Struß, A Schambach, B Tümmler
Ausgabe
Heft 06 / 2014
Seiten
–
Erschienen: 2014-06-10 00:00:00
ISSN
0934-8387
DOI
10.1055/s-0034-1376787

Zusammenfassung

Cystic fibrosis (CF) is the most common hereditary disease in the Caucasian population. The disease causing gene CFTR was identified in 1989. So far, more than 1,800 mutations have been identified in the CFTR gene [1], which codes for a cyclic adenosine monophosphate-regulated chloride channel. The malfunction of this channel leads to a progressive loss of function in the CF lung, which is the decisive factor for morbidity and mortality of most CF patients. Beside mucus accumulation in the airways, also an impaired phagocytosis in alveolar macrophages seems to play an important role in the pathogenesis of chroni…

Schlagworte

Adenosine Monophosphate Population Adenosine Chloride Database After Pneumologie