POLG Mitochondrial Disease Kills Prince Frederik Of Luxembourg At 22: Can It Be Treated? Know Symptoms And Who It Affects

Prince Frederik of Luxembourg, the youngest son of Prince Robert of Luxembourg and Princess Julie of Nassau, has died after a lifelong battle with a rare genetic disorder known as POLG mitochondrial disease. He was 22. Frederik died in Paris on March 1, his family announced in a statement on the website of the POLG Foundation, which Frederik founded in 2022.

POLG is a “genetic mitochondrial disorder that robs the body’s cells of energy, in turn causing progressive multiple organ dysfunction and failure,” according to the foundation’s website. The prince died a day after Rare Disease Day, an international day of awareness for rare diseases. An estimated 300 million people live with a rare disease and Frederik succumbed to one.

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What is POLG?

POLG disease causes a wide range of symptoms and affects so many different organ systems. It is very difficult to diagnose and treat. It causes progressive multiple organ dysfunction and failure.

According to POLG Foundation, mitochondria are the power plants of the cell, converting the food we eat into the kind of energy our bodies need to function. Besides being critical, one peculiarity of these organelles is that they contain their own DNA which, to replicate, requires an enzyme (polymerase γ; POLG) encoded in the host cell’s POLG and POLG2 genes. Mutations in those genes impair efficient replication of the mitochondria DNA.

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What are the symptoms of POLG and who does it affect?

POLG symptoms, which can be mild to severe, most often include ophthalmoplegia, muscle weakness, epilepsy and liver failure. symptoms can start from early childhood to adulthood.

Can POLG be treated?

For all patients with PolG disorders, the future is deeply uncertain. There are no effective treatments, let alone a cure that might save them. Every day they witness their own degeneration and progressive loss of function, a harrowing life experience.