January 30, 2011, Updated September 12, 2012

Israeli scientist, Dr. Yosef Shiloh, has been chosen by the American Association for Cancer Research (AACR) as this year’s winner of the prestigious Clowes Award. The Tel Aviv University professor is the first Israeli researcher to win the coveted prize.

Shiloh is renowned for his research on ataxia telangiectasia (A-T), a rare, neurodegenerative, inherited disease that affects many parts of the body and causes severe disability.

“Professor Shiloh is an international leader in his field and an extraordinary scientist,” AACR director Dr. Margaret Foti wrote in the explanation for awarding him the prize. “His work has launched a scientific revolution and opened up new horizons in the understanding of how the living cell copes with DNA damage, which is among the main factors in cancer.”

The AACR is considered to be the most important organizations in the world for targeting the prevention and cure of cancer by promoting research, education and collaboration. Shiloh will also receive a $10,000 grant, and will address the AACR in April.

Shiloh, who is also an Israel Cancer Research Fund (ICRF) recipient, began exploring A-T in 1977, after meeting a family from the Negev whose four children suffered from the disease. In Israel, the A-T disease affects people of North African origin, the Palestinian and Bedouin communities.

Shiloh’s work focused on the mechanisms that enable the cell to overcome DNA damage caused by various environmental factors such as radiation, chemicals in food and pollutants.

This damage is common in healthy people too, but it is repaired by complex defense mechanisms, which are vital to preserving cell life and preventing cancer. These defense mechanisms are not activated in A-T patients.

Over the course of his research career, Shiloh made several discoveries that contributed to understanding the disease, including discovering the defective gene that causes it. This enabled detection of the disease in the early stages of pregnancy and paved the way to understanding its basis – a defective DNA damage response.

“Our great hope is that understanding the complex defense mechanism will enable new ways of treating the disease and other diseases caused by failures in our defense from DNA damages,” Shiloh said.

In a letter to the Israel Cancer Research Fund, Shiloh said that he is frequently asked – even by doctors – why he has devoted his career to such a rare genetic disorder. He wrote: “Patients with rare diseases don’t care about the frequency of their disease; it’s THEIR disease, it makes their lives and the lives of their families extremely difficult. Hence, it requires our attention – and investment.”

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