Bristol, October 17, 2025
News Summary
A new study reveals that the longevity gene BPIFB4 may slow down heart aging and improve heart function in mice with Hutchinson-Gilford Progeria Syndrome. Researchers found that a single injection of BPIFB4 improved heart tissue health and reduced aging markers in progeria models. This research offers hope for developing gene therapies targeting heart-related symptoms of this rare genetic condition, which accelerates aging in children.
City, October 26, 2023 – A recent study published in Signal Transduction and Targeted Therapy reveals that the longevity gene BPIFB4 could play a pivotal role in slowing down heart aging, particularly in model mice mimicking Hutchinson-Gilford Progeria Syndrome (HGPS). This groundbreaking research is the first to indicate that a longevity gene from individuals with extended lifespans may help combat the heart-related symptoms associated with this rare genetic disorder.
Hutchinson-Gilford Progeria Syndrome is a genetic condition that accelerates aging in children, caused by a mutation in the LMNA gene. This mutation results in the overproduction of a toxic protein called progerin, which disrupts the structural integrity of cell nuclei and leads to early aging signs, especially within the heart and blood vessels. Individuals diagnosed with HGPS face severe medical challenges and typically succumb to complications related to heart function by their teens. Sammy Basso, known as the oldest progeria patient, passed away at the age of 28 on October 24, 2024.
Currently, the only FDA-approved treatment for HGPS is a drug called lonafarnib, which effectively reduces progerin accumulation in patients. There is an ongoing clinical trial investigating the efficacy of combining lonafarnib with another drug known as Progerinin to enhance treatment outcomes.
The research was carried out by scientists from the Bristol Heart Institute and IRCCS MultiMedica in Italy, focusing on the BPIFB4 gene, previously associated with maintaining heart health through the aging process. Through experimentation, it was found that progeria mouse models exhibited early heart dysfunction similar to that observed in affected children, establishing a base for further study. A single injection of the LAV-BPIFB4 gene yielded notable results, improving diastolic function in the heart of progeria mice, minimizing damage to heart tissue indicated by fibrosis, and reducing the number of aged cells present.
Moreover, the application of the BPIFB4 gene promoted the growth of small blood vessels, which is essential for heart tissue health and function. When researchers examined human cells derived from progeria patients, they observed that introducing the LAV-BPIFB4 gene alleviated signs of cell aging and fibrosis, all while not directly affecting the levels of progerin present within the cells. This suggests that the gene’s mechanism may involve helping cells better manage the toxic impact of progerin instead of simply eliminating the problematic protein.
Researchers interpret these findings as a sign of hope for developing new treatment approaches that focus on the biology of healthy aging rather than exclusively targeting defective proteins. The significance of this research lies in its potential to shape innovative treatment strategies that could mitigate cardiovascular damage in those afflicted with progeria.
Moving forward, therapeutic applications may include gene therapy or advanced methods to deliver proteins or RNA that target the longevity gene BPIFB4 effectively, potentially opening new avenues in treating this devastating condition.
FAQs
What is Hutchinson-Gilford Progeria Syndrome (HGPS)?
HGPS is a rare genetic disorder that causes rapid aging in children due to a mutation in the LMNA gene, leading to excessive production of a toxic protein called progerin.
What are the current treatments for HGPS?
Currently, the only FDA-approved treatment for HGPS is lonafarnib, which helps reduce progerin accumulation. A clinical trial is also ongoing to test lonafarnib in combination with another drug called Progerinin.
How does the LAV-BPIFB4 gene improve heart function in progeria models?
The LAV-BPIFB4 gene improved diastolic heart function in progeria mice, reducing heart tissue damage, decreasing aged cell presence, and enhancing the growth of small blood vessels.
Key Features
| Feature | Description |
|---|---|
| HGPS Overview | A rare genetic disorder causing rapid aging in children. |
| Treatment Options | FDA-approved lonafarnib and ongoing clinical trials for combination therapy. |
| BPIFB4 Gene | Gene shown to improve heart health and longevity in aging models. |
| Research Findings | BPIFB4 enhances heart function, reduces fibrosis, and promotes blood vessel growth. |
Deeper Dive: News & Info About This Topic
HERE Resources
Additional Resources
- The Washington Post
- Wikipedia: Hutchinson–Gilford Progeria Syndrome
- Nature
- Google Search: lonafarnib
- Medical Xpress
- Google Scholar: BPIFB4 gene
- Orphanet Journal of Rare Diseases
- Encyclopedia Britannica: Progeria
- National Geographic
- Google News: Longevity Gene
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