Sammy Basso, a Venetian biologist
suffering from
the fast-aging genetic disease progeria, died Saturday night at
the age of 28, his family siad Sunday.
He was the longest-living patient with this pathology.
(Hutchinson-Gilford syndrome) which causes
premature aging, and had campaigned tirelessly to raise
awareness of it.
The disease does not alter the mind, which remains the only
indicator of the true age of the patient.
Basso died late Saturday evening after being struck by a sudden
illness while he was in a restaurant in Treviso with his family.
A graduate in Natural Sciences with a thesis dedicated to the
existence of therapies to slow the progression of his pathology,
he had been appointed a Knight by President Sergio Mattarella.
He had also founded the Italian Progeria Association Sammy
Basso.
Progeria is a specific type of progeroid syndrome, also known as
Hutchinson-Gilford syndrome or Hutchinson-Gilford progeroid
syndrome (HGPS).
A single gene mutation is responsible for causing progeria.
The affected gene, known as lamin A (LMNA), makes a protein
necessary for holding the cell nucleus together.
When this gene mutates, an abnormal form of lamin A protein
called progerin is produced.
Progeroid syndromes are a group of diseases that cause
individuals to age faster than usual, leading to them appearing
older than they actually are.
People born with progeria typically live until their mid- to
late-teens or early twenties.
Severe cardiovascular complications usually develop by puberty,
later on resulting in death.
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