Chronic liver disease is a major complication of cystic fibrosis (CF) and factors other than the deletion of ΔF508 (which causes CF) may control the development of liver damage. In May Journal of Medical Genetics Gabolde and colleagues from Hôpital Robert Debré, Paris report that an independent genetic factor — the mannose binding lectin (MBL) gene — influences the severity of chronic liver disease in CF patients.

Gabolde et al studied the association between the allelic variants of MBL and the presence of liver cirrhosis among a homogenous homozygous ΔF508 population of 216 patients with CF. Denaturating gradient gel electrophoresis analysis showed that the presence of cirrhosis was significantly associated with a mutated MBL genotype.

Immunodeficiency characteristically associated with MBL variants could increase the hepatotoxic damage from viruses and may therefore facilitate the degradation of the liver in these patients.

Interested in reading more?

Become a Member of

Receive full access to more than 35 years of archives, as well as TS Digest, digital editions of The Scientist, feature stories, and much more!
Already a member?