Paradoxical role of proteinase inhibitors in cystic fibrosis

-antichymotrypsin is associated with less severe pulmonary disease in patients with cystic fibrosis.

By | December 28, 2000

While free proteinases destroy lung tissue in cystic fibrosis, logic predicts that proteinase inhibitors should offer protection. However, for some time doctors have observed that a mild genetic deficiency in an inhibitor is actually associated with a better, rather than worse, outcome. In January's issue of Thorax, Dr Mahadeva and colleagues from the University of Cambridge support this observation with their new findings (Thorax 2001 56:53-58).

They screened 157 patients with cystic fibrosis and identified 10 with a plasma deficiency of α1-antichymotrypsin (plasma concentration <0.2 g/l). In a multivariate analysis these individuals had significantly less severe lung disease than those who had normal or raised levels of α1-antichymotrypsin. They concluded, paradoxical though it may be, that a deficiency of the inhibitor α1-antichymotrypsin is associated with less severe pulmonary disease in patients with cystic fibrosis.

Popular Now

  1. Thousands of Mutations Accumulate in the Human Brain Over a Lifetime
  2. Two Dozen House Republicans Do an About-Face on Tuition Tax
  3. 2017 Top 10 Innovations
    Features 2017 Top 10 Innovations

    From single-cell analysis to whole-genome sequencing, this year’s best new products shine on many levels.

  4. The Biggest DNA Origami Structures Yet
    Daily News The Biggest DNA Origami Structures Yet

    Three new strategies for using DNA to generate large, self-assembling shapes create everything from a nanoscale teddy bear to a nanoscale Mona Lisa.

FreeShip