Zusammenfassung
Die zystische Fibrose (CF) ist eine häufige autosomal rezessive Erbkrankheit, die durch Mutationen im Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gen hervorgerufen wird. Das CFTR-Gen kodiert einen Membran-gebundenen Chlorid Ionenkanal; Mutationen im CFTR-Gen führen zu einer gestörten Salz- und Flüssigkeitssekretion in verschiedenen Geweben. Die Symptome der CF können, selbst bei Geschwistern oder Zwillingen mit den gleichen CFTR-Mutationen, in ihrer Ausprägung sehr variabel sein. Neben Umwelteinflüssen wird der Verlauf der pulmonalen Erkrankung auch durch weitere genetische Faktoren, so genannte modifizierende Gene, beeinflusst. Das Verständnis der molekularen und zellulären Grundlagen der gefundenen Genotyp-Phänotyp-Assoziationen wird zum besseren Verständnis der Erkrankung beitragen und wird helfen neue Ziele für pharmakologische Interventionen bei der CF zu identifizieren.
Abstract
Cystic fibrosis is a common autosomal recessive disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The CFTR gene encodes a membrane-bound chloride ion channel. CFTR gene mutations cause alterations in fluid and salt secretion of various tissues. The CF phenotype is highly variable even in siblings and twins carrying the same CFTR mutations. The course of CF pulmonary disease is modulated by both environmental and genetic factors independent of CFTR. This review summarises association studies that focused on disease modifier genes in CF. Understanding the molecular and cellular basis of the genotype-phenotype associations will help to better understand the disease and to identify new targets for therapeutic interventions in CF.
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PD. Dr. Hartmut Grasemann
Zentrum für Kinder- und Jugendmedizin · Universitätsklinikum Essen
Hufelandstr. 55
45122 Essen, Germany ·
Email: hartmutg@hotmail.com
