Medical Research

Title

Researcher

Commenced

Completed        

Amount

Grant/Award

Investigation into the Pathogenesis of Primary Biliary Cirrhosis

Professor James Neuberger, Queen Elizabeth Hospital, Birmingham

October 1997

September 2000

£75,000

In conjunction with British Liver Trust

These studies have provided a robust way of isolating and maintaining in culture human biliary epithelial cells (the main site of damage in PBC) so that laboratory studies can be carried out to investigate further the relationship between AMA and PBC.

Establishment of PBC Database

Professor Oliver James, Freeman Hospital, Newcastle

1997

1998

£10,000

Raised by Members of the PBC Foundation

This allowed the creation of a national database. As well as allowing the formation of a database that will provide more information about people in the UK with PBC but also showed that doctors can work together effectively.

Improving the Assessment of Outcome in Primary Biliary Cirrhosis

Professor Ann Jacoby, University of Liverpool Professor David Jones, University of Newcastle

February 2000

January 2003

£128,400

Community Fund

Development of the Health-Related Quality of Life Measure, namely the PBC 40

Newly Discovered Microbial Mimics as Triggers of PBC

Professor Diego Vergani, Royal Free and University College Medical School, University College London

March 2000

August 2001

£5,000

Bequeathed by Edgar Ernest of Dorset

These studies looked at whether microbes (such as some bacteria) can confuse the body’s immune system so that instead of reacting with microbes, the immune system attacks similar targets in the liver. They showed that there may be cross-reaction with a number of bacteria, which could trigger PBC in some individuals.

The Role of the Pregnane X receptor in Primary Biliary Cirrhosis Pathogenesis

Dr Matthew C Wright, University of Aberdeen

October 2003

October 2006

£75,000

Raised by Members of the PBC Foundation

This study looked at a protein called PXR and studied whether activation of this inhibits inflammation in the liver.   Since PXR activation promotes hepatocyte growth and is also anti-fibrogenic, the PXR may be an excellent drug target for the treatment of chronic inflammatory liver disease and PBC.

Genetic Study into Primary Biliary Cirrhosis

Dr George Mells

Dr Graeme Alexander

Dr Richard Sandford

June 2008       In progress

£50,000

Raised by Members of the PBC Foundation

The PBC Genetics Study was a UK-wide initiative to establish the UKPBC Research Cohort, a cohort of more than 4000 PBC patients for population-level research, including large-scale genetic studies. DNA samples obtained from the UKPBC Research Cohort were used for a genome-wide association study of PBC in 2010 and an iCHIP study of PBC in 2012. The PBC Genetics Study has now evolved into the UKPBC Project, which is broader in its ambition to elucidate the biological origins of PBC.

Role of Biliary Senesence in Cholestatic Liver Disease

Dr Simon Rushbrooke &

Dr GJ Alexander

Norfolk & Norwich University Hospital

March 2012

In progress

£80,000

Bequeathed by

Barbara Greaves (Fellowship Award)

This programme is currently underway.