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By Jordan King
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Prince Frederik of Luxembourg died at the age of 22 due to a rare mitochondrial condition called POLG disease—Newsweek has rounded up what you need to know about the illness.
The Context
The European royal died in Paris on March 1, one day after "rare disease day," according to his father Prince Robert of Luxembourg's public announcement.
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Frederik, who was diagnosed with POLG at the age of 14, founded the POLG Foundation along with his family to improve research into the disorder.
It was on the POLG Foundation's website that Frederik's death was announced—his dad wrote: "This is the battle that Frederik fought, and this is the burden that he had to carry throughout his life. He always did so with grace and with humor. When we asked him if he wanted to create a foundation to find a cure and help others like him, he jumped at the opportunity.
"Though he always made it very clear that he did not want this dreadful disease to define him, he nonetheless immediately identified with and helped define the mission of The POLG Foundation."
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What Is POLG Mitochondrial Disease?
Prince Robert described the disease as "a genetic mitochondrial disorder that robs the body's cells of energy, in turn causing progressive multiple organs (brain, nerves, liver, intestines, muscles, swallowing and ocular function, etc.) dysfunction and failure."
"One might compare it to having a faulty battery that never fully recharges, is in a constant state of depletion and eventually loses power," he said.
Robert also described how rare diseases such as POLG mitochondrial disease are often difficult to recognize as "POLG disease causes such a wide range of symptoms and affects so many different organ systems."
The only treatments available are those which help people manage symptoms and there is no cure.
Sir Douglas Turnbull, a professor and member of The POLG Foundation Scientific Advisory Board, said: "I have looked after literally hundreds of patients with mitochondrial disease and there is little doubt that of all mitochondrial diseases, POLG deficiency is the worst. It is so relentlessly progressive, attacking so many different systems with sadly the same conclusion."
What Causes POLG?
POLG disease is caused by inherited mutations in the POLG gene—more than 200 disease-causing POLG mutations are known, according to the POLG Foundation.
These mutations "compromise the functioning of the enzyme responsible for mitochondrial DNA maintenance."
Symptoms differ depending on when the disease first emerges. For those with disease onset before the age of 12, the main symptoms include seizures, cognitive regression, motor impairment, cortical visual loss, feeding difficulties and liver dysfunction.
For those with disease onset between the ages of 12 and 40, the primary symptoms include seizures, impaired coordination and peripheral neuropathy.
What Is the Luxembourg Royal Family?
The Luxembourg royal family, officially known as the Grand Ducal Family of Luxembourg, plays a symbolic role in Luxembourg's governance which is actually run by elected officials—Luxembourg is a constitutional democracy.
The Grand Duke performs ceremonial duties, including formally appointing the elected prime minister, signing laws into effect and representing Luxembourg abroad.
The current monarch is Grand Duke Henri, who is the cousin of Frederik's father Robert.
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