Why Progeria Patients Grow Old Before Their Time

A high-throughput screening study has identified a drug that could help slow down aging in patients with progeria.

AsianScientist (Apr. 17, 2017) – A team from the Daegu Gyeongbuk Institute of Science and Technology (DGIST) has not only identified the pathway that causes premature aging in patients with Hutchinson-Gilford progeria syndrome (HGPS) but also found a drug that could slow aging in these patients. Their findings have been published in Aging Cell.

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 just 13 years as they age ten times faster than others.

In the present study, researchers have linked the activation of a protein called ROCK with the mitochondrial dysfunction associated with cell aging in HGPS.

The team first observed that the levels of reactive oxygen species increase as mitochondrial function diminishes in cells from HGPS patients. Using high-throughput screening, they found a drug called Y-27632 that could effectively control reactive oxygen species production and improve mitochondrial function.

Further research showed that Y-27632 inhibits the phosphorylation level of ROCK and increases the oxidative phosphorylation efficiency of mitochondria. The study also confirms that the drug restores mitochondrial function and induces the recovery of aging cells by reducing the nuclear membrane degeneration and genetic damage that are characteristics of HGPS patient cells.

“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,” said study corresponding author Professor Park Sang-Chul.

“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.”

The article can be found at: Kang et al. (2017) Chemical Screening Identifies ROCK as a Target for Recovering Mitochondrial Function in Hutchinson-Gilford Progeria Syndrome.


Source: Daegu Gyeongbuk Institute of Science and Technology; Photo: Shutterstock.
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