Getting To Know The Genetic Disorders Of PoLG, The Cause Of Prince From Luxembourg Dies At A Young Age
JAKARTA - Prince Frederik, son of Prince Robert and Princess Juliet in Luxemberg died at the age of 22 on March 1, 2025. He died from the mitochondria of PoLG he suffered.
Since the death of Prince Frederik, the mitochondria disease of PoLG has drawn the spotlight. This is known to be a rare genetic disease, which until now has not been found a cure to overcome it.
Quoted from The POLG Foundation, on Monday, March 10, 2025, PoLG disease is grouped as a genetic disease due to abnormalities that occur in mitochondria. This disorder is caused by mutations in the PoLG gene which function to replicate mitochondrial DNA.
Mutations that occur disrupt replication and repair of DNA, which cause a shortage of energy production in cells. One of the most prominent disturbances of this disease is the Alpers-Huttenlocher Syndrome (AHS), which is characterized by encephalopathy, seizures, and liver failure.
Not only that, but this disease also causes severe disorders such as liver dysfunction, myopati and hypotonia, weakening muscles that control eye movements. Muscle weakness in these eyes makes eyewear decrease (ptosis) and difficulty moving the eye.
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In addition, a person with a rare genetic disorder will also experience acidia or lose coordination of the body, neuropathy or nerve damage, and other neurological symptoms. These various symptoms appear depending on the type of PoLG suffered and their appearance also occurs in varying age.
Until now, there has not been a way and medicine to treat this disease so that patients can recover. What can be done in PoLG disorder patients is only to manage their symptoms, to improve their quality of life, but cannot heal.
Thus, someone who has been diagnosed with this disease can have a fatal impact and lead to death. Prince Frederik himself has had PoLG genetic disorders since birth, but was only diagnosed at the age of 14 when his symptoms were very clear. He underwent treatment to relieve symptoms for 8 years, until finally he died at the age of 22.