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Breakthrough Offers Hope for Children with Progeria: ‘Longevity genes’ Show Promise in Reversing Rapid Aging
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(Image: A compelling, ethically sourced image of a child, or a scientific illustration related to Progeria research. Avoid overly distressing imagery.)
A groundbreaking finding offers a beacon of hope for children suffering from Progeria, a devastatingly rare genetic disease that causes premature aging. researchers at the University of Bristol and IRCCS MultiMedica have identified “longevity genes” – found in individuals who live exceptionally long lives (over 100 years old) - that show potential in reversing the heart damage caused by this life-limiting condition. This research, published in Signal Transduction and Targeted Therapy, marks the first time a gene from long-lived people has been shown to slow down heart aging in a Progeria model.
What is Progeria?
Progeria, formally known as Hutchinson-Gilford Progeria Syndrome (HGPS), is an incredibly rare, fatal genetic condition characterized by the rapid onset of aging symptoms in children. It affects approximately 1 in 20 million live births. Children with Progeria typically appear normal at birth,but begin to exhibit signs of accelerated aging within the first two years of life. These signs include:
* Growth retardation: Slowed growth and smaller stature.
* Hair loss: Thinning hair and eventual baldness.
* Skin changes: Thin, wrinkled skin.
* Cardiovascular problems: The most critically important and life-threatening symptom, leading to heart disease and stroke.
* Stiff joints: Limited range of motion.
Most individuals with Progeria die in their teens, primarily due to complications from cardiovascular disease. However, advances in treatment and care have led to some individuals living into their twenties, like the late Sammy Basso.
The Genetic Root of Progeria: Progerin and LMNA
Progeria is caused by a mutation in the LMNA gene. This gene provides instructions for making a protein called lamin A, which is crucial for maintaining the structure of the cell nucleus – the cell’s control centre. The mutation results in the production of an abnormal protein called progerin.
Progerin is toxic to cells. It disrupts the normal structure of the nucleus, leading to premature cellular aging, especially affecting the heart and blood vessels. The accumulation of progerin causes progressive damage, ultimately leading to the severe cardiovascular problems that characterize Progeria.
Current Treatments and the Need for Innovation
Currently, the only FDA-approved treatment for Progeria is lonafarnib.This drug helps reduce the build-up of progerin, slowing down the progression of the disease. However, it doesn’t cure Progeria, and its effects are limited. A clinical trial is underway testing lonafarnib in combination with another drug, Progerinin, to see if the combination offers improved outcomes.
The search for more effective treatments is critical. This new research offers a promising option approach: harnessing the power of genes associated with exceptional longevity.
The ‘Longevity Gene’ Breakthrough: LAV-BPIFB4
Researchers, led by Dr. Yan Qiu and Professor Paolo Madeddu at the Bristol Heart institute, and Professor Annibale Puca’s team at IRCCS MultiMedica, focused on a specific “longevity gene” called LAV-BPIFB4. This gene was identified in centenarians – individuals who live to be 100 years or older. Previous studies have shown that LAV-BPIFB4 plays a
