Mechanism of aging recovery for progeria patients revealed

'Y-27632' drug inhibits the phosphorylation level of ROCK and increases the oxidative phosphorylation efficiency of mitochondria. 

DGIST's research team has identified a mechanism that can recover the aging of patients with Hutchinson-Gilford Progeria Syndrome (HGPS). DGIST announced that the Chair Professor Park SangChul of New Biology (Head of Well-Aging Research Center) and the research team led by Professor Lee YoungSam has discovered a drug that can improve the aging of HGPS patients and identified the mechanism of aging recovery by using the drug.

05 april 2017--HGPS represents one of premature aging syndromes. The patients with HGPS experience growth retardation as well as age-associated symptoms such as skin wrinkles, hair loss, visual impairment, and cardiovascular diseases. Their average life expectancy is 13 years as they age 10 times faster than others. When it comes to aging control, improvement of biological function and solutions for aging of HGPS patients have been big challenges in academia.
This study needs to be highlighted as the research team has identified the molecular causal relationship between ROCK protein activation and mitochondrial dysfunction in the progression of cell senescence for the first time in the world.
DGIST's research team noted that the level of reactive oxygen species increases when mitochondrial function diminishes during the progression fibroblasts of the HGPS patients. By performing high-throughput screening system, the team found 'Y-27632' as an effective agent to control reactive oxygen species and discovered that this drug is effective in improving mitochondrial function.
Mitochondria are intracellular organelles that play a key role in energy and metabolism in cells. It is known that mitochondrial dysfunction causes aging of cells as it increases level of active oxygen and decreases energy production efficiency.
The research team found that 'Y-27632' drug inhibits the phosphorylation level of ROCK and increases the oxidative phosphorylation efficiency of mitochondria. The study also confirms that the drug recovers mitochondrial function and induces the recovery of aging cells by reducing nuclear membrane degeneration and genetic damage that are characteristics of HGPS patient cells.
The Chair Professor Park SangChul stated "This study is significant as we have newly discovered the means to control aging. We have also identified the mechanism to recover the function of aging cell through inhibition and recovery of mitochondrial dysfunction due to aging." He added "We will continue to carry out studies that will extend the healthy lifespan of humans through the verification of the mechanism in aging animal models as well as progeny animal models."

More information: Hyun Tae Kang et al, Chemical screening identifies ROCK as a target for recovering mitochondrial function in Hutchinson-Gilford progeria syndrome, Aging Cell (2017). DOI: 10.1111/acel.12584


Provided by Daegu Gyeongbuk Institute of Science and Technology

The rare aging disease, Progeria, linked to aging in the general population

Study results demonstrate progerin increases in blood vessels as we age

BOSTON, MA , 08 sept 2010– Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS), is a rare, fatal genetic disease characterized by an appearance of accelerated aging in children. All children with Progeria die of the same heart disease that affects millions of normal aging adults (atherosclerosis), but instead of occurring at 60 or 70 years of age, these children may suffer heart attacks and strokes even before age 10, and the average age of death is 13 years. Finding treatments and a cure for Progeria may provide clues to preventing or treating heart disease in the general population.

On August 26, 2010, Arteriosclerosis, Thrombosis, and Vascular Biology electronically published, ahead of print, the results of a study comparing Progeria, and typical cardiovascular aging, entitled "Cardiovascular Pathology in Hutchinson-Gilford Progeria: Correlation With the Vascular Pathology of Aging". The study found that progerin, the abnormal protein that causes Progeria, is also present in the vasculature of the general population and increases with age.

Researchers examined cardiovascular autopsies and progerin distribution in two patients with Progeria along with a group of 29 individuals without Progeria. Between the ages of one month and 97 years, researchers found that progerin in individuals without Progeria increased an average of 3.3 percent per year in the coronary arteries.

"We found similarities between many aspects of cardiovascular disease in both Progeria and the atherosclerosis that affects millions of people throughout the world" said Dr. Leslie Gordon, senior author of the study, and Medical Director of The Progeria Research Foundation. "We also found progerin in cardiac blood vessels of people without Progeria. The fact that progerin is present at all tells us that there may be a tie between the heart disease of Progeria and the heart disease that affects us all as we age. "

This study supports the possibility that progerin is a contributor to the risk of atherosclerosis in the general population, and merits examination as a potential new element influencing vascular health with aging.

"By examining one of the rarest diseases in the world, we are gaining crucial insight into a disease that affects millions of people worldwide. Ongoing research has the potential to have a significant impact on our understanding of heart disease and aging," said Dr. Gordon


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The Progeria Research Foundation (PRF) was established in 1999 to find the cause, treatment and cure for Progeria – a rapid aging disease that causes children to die from heart disease or stroke at an average age of 13. In the past 10 years, research conducted in partnership with PRF has identified the gene that causes Progeria and possible treatments. PRF is now funding first-ever Progeria clinical drug trials, currently underway at Children's Hospital Boston. To learn more about Progeria and what you can do to help, please visit progeriaresearch.org