Death of Sammy Basso: in Montpellier, the quest for anti-aging recipes from progeria, a disease of premature aging

Sammy Basso, en 2015, avec l’animateur italien Carlo Conti. MAXPPP – ETTORE FERRARI

Italian Sammy Basso, who died last weekend at the age of 28, has put a face on progeria, a disease that results in premature aging. Jean-Marc Lemaitre, a research director at the IHU (University Hospital Institute) of the Montpellier University Hospital, who devotes his work to rejuvenation, explains how research on this very rare pathology serves the fight against aging.

In 2011, Jean-Marc Lemaitre was the first scientist in the world to reprogram centenarian cells into embryonic stem cells. He is the author of “Guérir la vieillesse”, published in 2022.

Jean-Marc Lemaitre and his teams are working on genetically mutated mice suffering from progeria. Midi Libre – SYLVIE CAMBON

Italian Sammy Basso, who died last Saturday, has put a face to progeria, or Hutchinson Gilford syndrome, a disease that results in premature aging. It is a favorite subject for a researcher who, like you, devotes his life to finding the keys to rejuvenation ?

There are common denominators in progeria research and in our research. It is a very rare disease, particularly dramatic, the most dramatic among the genetic diseases of accelerated aging in men. There are many. Werner syndrome, for example (Editor's note: a disease that appears between the ages of 20 and 30 and affects one in 200,000 people) or the so-called “moon children” disease, and Rothmund-Thomson syndrome…

Progeria is caused by a mutation in the LMNA gene encoding lamins A/C that leads to the accumulation of a protein, progerin. It is an aberrant protein. Progeria is a very rare disease, caused by a mutation on a single nucleotide of DNA, which affects one person in 8 million. There are barely three or four patients in France.

The protein is not synthesized in the brain, and in these individuals, the brain does not age at the same rate as the body; Sammy Basso had also done studies.

Your research focuses on progeria?

A Spanish colleague, who works with Marseille professor Nicolas Lévy, whose team discovered the gene for the disease in 2003, provided us with animals whose LMNA gene had been modified. Suffering from progeria, they live six months instead of three years.

We are also working on human cells.

We are working on both DNA reprogramming and the destruction of senescent cells (Editor's note: cells that can be described, to simplify, as “waste”), which are involved in aging. This destruction alone could increase life expectancy by 30%. There are clinical trials in the United States, and perhaps patients with progeria will benefit from these treatments.

“With “natural” aging, this protein appears”around age 60″

Because physiological “natural” aging resembles, at one point in our life, what happens when we have progeria ?

With "natural" aging, this protein actually appears, around the age of 60.

What are the hopes for treatment ?

An anticancer drug, Lonafarnib, an inhibitor that prevents the harmful protein from acting, would allow us to gain four years of life, that's + 30 %. More generally, all the strategies implemented against aging are useful in the fight against premature aging.

In addition to the efforts of teams of scientists, the Progeria Research Foundation, an American foundation, is very active and manages to raise ten million dollars each year for research.

Are there other avenues ?

Today, there is a lot of talk about Metformin, an antidiabetic molecule that would also be interesting against aging.