CNN – 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.
Frederik was diagnosed with POLG at age 14, the family said.
Because the disease causes such a wide range of symptoms and affects many different organ systems, it can be difficult to diagnose.
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.
“As is the case for 300 million people like Frederik worldwide, these diseases are usually hard to recognise even by physicians, and patients’ families may never know what they are suffering from as they may only be identified very late in their progression,” his father, Robert, said …
United Mitochondrial Disease Foundation, PITTSBURGH, PA – PolG disease is a mitochondrial disorder caused by mutations in the POLG gene.
It typically affects multiple organs, primarily the brain, nerves, muscles, and liver, and can affect vision due to involvement of brain structures.
It is also one of the most common inherited mitochondrial disease.
Up to 2% of those of Northern European decent may carry disease-causing POLG mutations, and the frequency of PolG disease is estimated to be 1 in 10,000.
Mutations may be autosomal recessive (meaning two mutations are required for disease expression) or autosomal dominant (meaning only one mutation is required for disease expression).
Signs of the disease can appear anywhere between infancy and late adulthood. Symptoms vary widely among affected individuals. source.
