Zusammenfassung
Cystic fibrosis (CF), the most-common lethal hereditary disease in the white population, is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The mutation that is most frequently responsible for the disease, DeltaF508, causes misfolding and retention of the CFTR protein in the endoplasmic reticulum. This leads to a series of cellular dysfunctions and ...
Zusammenfassung
Cystic fibrosis (CF), the most-common lethal hereditary disease in the white population, is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The mutation that is most frequently responsible for the disease, DeltaF508, causes misfolding and retention of the CFTR protein in the endoplasmic reticulum. This leads to a series of cellular dysfunctions and results in a multi-organ disease. In a recent report, Egan et al.((1)) demonstrated that curcumin, a non-toxic natural product and major constituent of turmeric spice, corrected the CF defects in DeltaF508 CIF mice. This paper aroused a lot of attention and hopes were raised that curcumin might produce similar effects in human, giving an efficient treatment for most CF patients. However, skepticism is growing since subsequent studies fail to reproduce these initial exciting results. Thus, although herbal medicines and dietary supplements can be desirable alternatives to classical pharmacological compounds, their efficacy needs careful evaluation both in vivo and ex vivo. (C) 2004 Wiley Periodicals, Inc.
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