Hungarian TPI Research by Professor Susan Hollan

Last updated March 15th 1998

Professor Susan Hollan is a Member of the Hungarian Academy of Science at the National Institute of Haematology, Blood Transfusion and Immunology in Budapest.

Since we first contacted her she has been very supportive and helpful, and in the very early days when James's story broke to the national media, she was prepared to allow the UK press to interview the Hungarian TPI Brothers. Unfortunately the media did not take up her offer!

Professor Hollan has been researching TPI deficiency ever since the 2 Brothers were diagnosed in 1988. In that time she has built up a collaboration with TPI experts in London, Japan, America and France. We asked her to put together a proposal for further TPI research under her direction. Her proposal was accepted by the trustees of 'The James Stewardson Research and Welfare Trust for Children with TPI' at a cost of 15,000. Professor Hollan's research project was granted another 20,000 in January 1997 and another 20,000 in February 1998. The 'Aims and Objectives' of her Research Project are:-

1) To assess the genetic and cellular, structural and functional difference in severly affected TPI deficient individuals between those with neurological symptoms and life threatening outcomes and those without neurological impairment and benign clinical course.

2)To asses the changes in the lipiid and protein constituents of the cellular membrane from TPI deficient families and define the role of these changes for the variable effect of TPI deficiency on brain function.

3) A better understanding of the membrane alterations in TPI deficient cells may facilitate in finding the best ways for enzyme replacement approaches to reverse the clinical effects of TPI deficiency.

The proposal included confirmation of complete collaboration between the Hungary and London teams and that blood samples would be exchanged by both teams.

Professor Hollan met Professor Bellingham and Dr Roopen Arya at a conference in Istanbul in late 1995, where they discussed TPI and how they could collaborate together. She again met Roopen at the Annual Meeting of the American Society of Haematology in December 1995. Roopen gave a presentation on some of his TPI deficiency findings at the convention, which was very well received.

Professor Hollan was in London in July 1996, which co-incided with James being in London at Kings for treatment and met James.

Professor Hollan will periodically update this page with her TPI research progress.

Professor Hollan published a TPI paper in September 1997 detailing her progress in researching the differences of the two TPI deficient Hungarian brothers. Research Paper