GOLD Annual General Meeting, 2008

GOLD’s Annual General Meeting, 2008 will take place during the 10th International Symposium on Mucopolysaccharide and Related Diseases, June 26-29, 2008. Vancouver, Canada.

Date:               Saturday June 28th
Time:              5.00pm – 6.30pm
Venue:            Junior Ballroom, Sheraton Vancouver Wall Centre, Vancouver, Canada.

We will welcome the attendance of non-members, but as space may be limited, preference will be given to GOLD’s member organisations. Please complete the reply form notifying GOLD of your intention to attend to AGM to assist our planning. If you have any queries, please contact GOLD. If you are unable to attend, you may nominate another person to represent your organisation at the meeting. GOLD regrets that it is unable to provide travel bursaries for members to attend the meeting.

Guest Speaker: Dr William Sly, MD PhD

We are pleased to announce that our guest lecturer will be Dr William Sly, Alice A. Doisy Professor and Chairman of Biochemistry and Molecular Biology, Department of Biochemistry and Molecular Biology, St. Louis University School of Medicine.

Each year, four members of the Management Council stand down by rotation. This year these members are: Ms Rhonda Buyers, Prof Tim Cox, Prof Roberto Giugliani and Dr Maurizio Scarpa. All are eligible for re-election. Calls for nominations will take place in April.

Members of GOLD will shortly be receiving a call for nominations and details of the election process for the 4 available places on the Management Council of GOLD. If there are more nominees than places available, a ballot will be held.

On behalf of the Management Council, I look forward to welcoming you to GOLD’s Annual General Meeting

Ann Hale

Executive Director, GOLD


Dr William Sly is best known for his contribution to research on lysosomal storage diseases and the mechanisms by which cells target enzymes to lysosomes. Dr Sly described the first patient with β-glucuronidase deficiency, a mucopolysaccharide storage disease now known as MPS VII or Sly Syndrome. Studying β-glucuronidase uptake and recognition, Dr Sly discovered that lysosomal enzymes contain mannose 6-phosphate residues that act as targeting signals for receptors which transport the enzymes from their sites of synthesis in the cell to their site of action in the lysosomes.

For this discovery he was elected to the National Academy of Sciences in 1989. Discovery of a second recognition system for β-glucuronidase on macrophages led to identification of the mannose receptor, studies which provided the rationale for others to develop mannose-targeted enzyme replacement in Gaucher Disease, now a successful clinical therapy. Dr Sly’s group cloned the human β-glucuronidase gene and characterised the mutations underlying Sly Syndrome, and also cloned and characterised the human IGFII/Man 6-P receptor that transports enzymes to lysosomes. For his studies elucidating the mechanism of lysosomal enzyme transport, he shared the Passano Award with Stuart Kornfeld of Washington University in 1991.

Working with collaborators at the Jackson Laboratory, he characterised the mouse model of Sly Syndrome and used this model to study the effects of enzyme therapy, bone marrow transplant and gene therapy for β-glucuronidase deficiency. Their work popularised the MPS VII mouse model which is now widely used in developing experimental approaches to gene therapy.

Dr Sly has been the recipient of numerous awards and honours, including election to The National Academy of Sciences. He is a member of numerous societies and on the editorial boards of many journals and other publications. Dr Sly was a founder member of GOLD and retired from the Management Council last year.



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