Interesting new lead

[Guest guest]

At the outset I'd like to apologize in advance if this post is too scientific. But I'm always on the look out for new leads in the PSC area, and this one caught my attention. It's a thesis from Uppsala University:

Author:

Ardesjö, Brita

Title:

Autoantigens in Inflammatory Bowel Disease and Primary Sclerosing Cholangitis

Department:

Uppsala University, Medicinska vetenskapsområdet, Faculty of Medicine, Department of Medical Sciences

http://publications.uu.se/theses/abstract.xsql?dbid=8677

In a nutshell it describes some new autoantibodies identified in PSC patients. The autoantibody that is most interesting is an antibody against a protein called Pdzk1. This is a protein that regulates the cystic fibrosis transmembrane conductance regulator (CFTR) protein that is responsible for bicarbonate excretion into the bile. You will recall that the liver disease associated with cystic fibrosis is very similar to primary sclerosing cholangitis, and that some researchers have shown that the CFTR gene may be defective in some PSC patients. Recent studies with children with PSC have intriguingly shown impaired CFTR activity without any obvious mutations in the CFTR gene, indicating that a protein regulating CFTR may be affected in these patients. Perhaps development of antibodies against Pdzk1 could somehow result in impaired CFTR activity, disrupting bile flow?

Dave

(father of (23); PSC 07/03; UC 08/03)