New study could offer protection to patients with rare genetic disease

Category: Health published by Syed Qayam Ali published: Feb. 24 2019, 11:44 am IST Update: Feb. 24 2019, 11:44 am IST

Washington: A new study from the University of Bath reveals that skin cells taken from patients with a rare genetic disorder are ten times more sensitive to ultraviolet A (AVA) damage during laboratory tests only those from a healthy population.

The experts believe that the work, which involves the design of a brand new molecule that could be added to sunscreen, could benefit patients with Friedrich ataxia (FA), as well as other disorders characterized by mitochondrial iron overload, including Wolfram Syndrome and Parkinson's Disease, where UVA rays from the sun can pose special problems.

When cells are exposed to UVA rays, damage to cells can be aggravated by excess free iron in the mitochondria, source of "free radicals," including reactive oxygen species (ROS ), which can damage DNA, proteins and fats – increasing the risk of cell death and cancer.

Patients with AF have high levels of free iron in their mitochondria. New research shows that this excess of free iron makes the skin cells of these patients ten times more susceptible to UVA damage.

Scientists have custom built a molecule that acts like a claw to pick up excess iron particles in the mitochondria, thus preventing them from amplifying the damage caused by UVA. The goal of the researchers is to see this molecule added to sunscreens to enhance their protective effect against UVA rays.

Dr. Charareh Pourzand, lead author of the study, said, "One of the main functions of mitochondria is to produce energy, and a sufficient amount of iron is essential to their functioning.

Pourzand added: "Friedreich's ataxia is an example of 'mitochondrial iron overload'. Our results, which should apply to people's skin (in vivo), suggest that patients could be 10 times more sensitive to UVA. The damage that you and I would suffer in our skin as a result, for example, of 2.5 hours exposure to solar UVA would be 4 to 10 times greater for a patient with FRDA. "

According to Pourzand, an excess of iron in the mitochondria means more reactive oxidative species and more damage to the cellular constituents, compromising cellular functions. This situation leaves the cells more susceptible to subsequent oxidative damage, especially due to environmental factors such as the UVA rays of the sun.

"We hope that our discoveries can finally be pbaded on to people to give them a better quality of life and that we can inspire other researchers to follow these leads. We are very grateful to our sponsor, the Biotechnology and Biological Sciences Research Council (BBSRC), for making this project feasible, "said Pourzand.

The team is now looking to continue research on the chelator with an in vivo murine model of the disease.

Source: ANI