 Researchers looked at the role of the BLM gene |
A rare condition has given scientists clues about how cells protect themselves from becoming cancerous. People with Bloom's syndrome, who lack a key gene, are at a high risk of developing cancer.
Cancer Research UK scientists have found how the gene helps to stop normal cells becoming cancerous.
They said the research, published in the magazine Nature, could help in developing ways of stopping cancer in its earliest stages.
People with Bloom's, who have inherited faulty versions of a gene called BLM, tend to have a small body size, sun-sensitive facial reddening, infertility, and a inefficient immune system. The mutation responsible for the condition is most common among Ashkenazi Jews, where two in every 100,000 are affected. It is much less common in other populations.
Because they are highly prone to developing cancer, they are monitored for the disease from a young age.
Scientists knew the BLM gene played a role in stopping normal cells from becoming cancerous, but did not know how.
Cells are designed to be able to repair their DNA when it gets damaged. But the repair process is imperfect and errors can lead to cancerous growth.
'No protection'
Cancer Research UK scientists at the Oxford Cancer Centre have found that, when it is working properly, the BLM gene prevents potentially cancer-causing errors during the DNA repair process.
Chromosomes wrap around each other in order to exchange lost information, and BLM helps them unwrap.
When BLM does not function, the chromosomes do not unwrap properly and errors occur.
People with Bloom's are prone to cancer because they do not have this protection.
Professor Ian Hickson, who led the research: "People with Bloom's syndrome have no working copy of the BLM gene. We have managed to unravel why that gene is so important to preventing cancer.
"It stops one of the cell's own DNA repair processes from causing potentially dangerous errors.
"Without BLM, the cell's repair machinery becomes its own worst enemy."
Professor Robert Souhami, Cancer Research UK's director of clinical and external affairs, said: "Studies into rare inherited conditions such as Bloom's syndrome can give us unique insight into cancer in all its forms.
"This study offers us an intriguing understanding of one of the fundamental processes involved at the very beginning of a cancer's development.
"The more we understand about the steps leading to cancer, the closer we will be to modifying those processes to stop the disease in its early stages."
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The more we understand about the steps leading to cancer, the closer we will be to modifying those processes to stop the disease in its early stages 
