Canadian Association for Familial Ataxias (ACAF)

Claude St-Jean Foundation

Friedreich's Ataxia

M.J Stones
Special to the Globe and Mail; Globe and Mail Archives

Claude St-Jean 1952-2006

Engaged in a media-awareness campaign ever since, the ACAF remains at the forefront of ataxia research. Dr. Massino Pandofo, who in 1996 was credited in identifying the Friedreich's ataxia gene, says it was Mr. St-Jean's single-minded determination that had fuelled the groundbreaking discovery.

"Thanks to his effort, since the 1970, many fine researchers from all over the world became interested in the disease," says Dr. Pandofo, who at the time was professor of neurosurgery at McGill University. "I directly witnessed Claude St-Jean's constant activity in supporting FA research and awareness, despite the advanced stage of his disease.
In a subsequent finding, Dr. Pandolfo and his team went even further and pintpointed how iron accumulates in cell that lack the frataxin gene. In a healthy cell, the gene produces a protein that regulates iron, which the cell uses to make energy out of fuel and oxygen. If the gene of the chemical react with oxygen and put stress on the cell. Eventually it dies.

During his lifetime, Mr. St-Jean raised about $6 Million for ataxia research. Not only was he a superb fundraiser, but he also played the part of the selfless test-patient, submitting himself to endless physical examinations and to new and often unproven medicines and procedures.

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