Died last weekend at the age of 28, Italian Sammy Basso put a face to progeria, a disease which results in premature aging. Director of research at the IHU (University Hospital Institute) of Montpellier University Hospital, Jean-Marc Lemaitre, 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 century-old cells into embryonic stem cells. He is the author of “Healing Old Age”, published in 2022.
Midi Libre – SYLVIE CAMBON
The Italian Sammy Basso, who died last Saturday, put a face to progeria, or Hutchinson Gilford syndrome, a disease which results in premature aging. Is it 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 our research. It is a very rare, particularly dramatic disease, the most dramatic among the genetic diseases of accelerated aging in humans. There are many. Werner syndrome, for example (Editor’s note: a disease which appears between the ages of 20 and 30 and which affects one person in 200,000) or the so-called “children of the moon” disease, and the Rothmund-Thomson syndrome…
Progeria is due to a mutation in the LMNA gene encoding lamins A/C which leads to the accumulation of a protein, progerin. It is an aberrant protein. Progeria is a very rare disease, caused by a mutation in a single nucleotide of DNA, which affects one in 8 million people. 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 carried out studies.
Does your research focus on progeria?
A Spanish colleague, who works with Marseille professor Nicolas Lévy, whose team discovered the disease gene in 2003, provided us with animals whose LMNA gene has been modified. Affected by progeria, they live six months instead of three years.
We also work on human cells.
We are working on both the reprogramming of DNA 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 progeria patients will benefit from these treatments.
“With “natural” aging, this protein appears around the age of 60”
Because “natural” physiological aging resembles, at a moment in our lives, 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 which prevents the harmful protein from acting, would save four years of life, that’s +30%. More generally, all the strategies put in place 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, we talk a lot about Metformin, an antidiabetic molecule which would also be useful against aging.
