Genes, health and inbreeding in an Italian village

Jennifer Dorian:

In the foothills of the Italian Alps is a tiny village that may hold a clue to a medical mystery that has challenged researchers for centuries. Despite indulging in an artery-clogging diet that could make even an Italian grandmother cringe, the 400 or so residents of tiny Stoccareddo, Italy, have virtually no heart disease or diabetes, and often live into their 90s.

While at first glance nothing seems to be unusual about the town, a closer look reveals almost everyone is related and shares the same last name of Bau (pronounced Bow-ooh). According to Amerigo Bau, the unofficial town historian, the first Bau family arrived in Stoccareddo from Denmark about 800 years ago and ever since, Baus have been marrying Baus.

"It happened because the town was in the mountains," explains Amerigo Bau. "It was isolated, and so the likelihood of marrying another Bau was quite strong."

Most Baus tended to marry more distant relatives and not first cousins, which can cause genetic defects.

Recently, the claim to fame for the residents of Stoccareddo has come to rest with their mysterious good health. Dr. Uros Hladnik, a genetic researcher at the Baschirotto Institute for Rare Diseases (B.I.R.D Foundation) in Vincenza, Italy is studying why the Baus of Stoccareddo seem to be able to eat fatty meats, cheeses and cream sauces without suffering the most serious consequences.

Preliminary results have found cholesterol levels of LDL — the so-called "bad" cholesterol — that are much lower than those of most Italians, and HDL levels — the so-called "good" cholesterol — that are much higher.

"They seem to be protected," Hladnik said. "Maybe the Baus have something that allows them to eat cholesterol."

But their good health may ultimately have less to do with their genetic code and more to do with their zip code. The air and water in Stoccaredoo are clean, and even today the town is still relatively isolated from many of the pressures and stresses of the outside world.

"Apart from the research that's going on, we believe that we have two genes that contribute to a better life: the good air that we breathe and the happiness that you breathe in the town," Amerigo Bau said.

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