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I magine youth, full of energy and wonder, coupled with a disease that weakens your muscles and turns your entire nervous system against you. This is the reality of being diagnosed at a young age with a PolG-related mitochondrial disease, a debilitating chronic and often fatal illness that was once considered rare but now, thanks to advancements in genetic testing, is more commonly known. Today, mitochondrial diseases (or “MITO”) affect around one in every five thousand people. Their profound implications are known well by Frederik de Nassau, the son of H.R.H. Prince Robert of Luxembourg, who was diagnosed with a mitochondrial disease. After extensive research, Prince Robert and his wife, Princess Julie, discovered the cause of their youngest son’s illness, and the difficult road ahead was revealed. “We spent a lot of time living in hospitals with our son and he had about eleven different surgeries over a short period of time,” Prince Robert told Sotheby’s.
That experience culminated in a desire to help those with MITO, by forming new partnerships with an array of researchers, laboratories and companies. Now, on 21 May, 2022, Prince Robert, who is also the entrepreneurial Chairman and CEO of the prestigious wine company Domaine Clarence Dillon — which includes Chateaux Haut Brion, La Mission Haut Brion and Quintus as part of its enterprise — is selling wine from his personal cellars to fund research into the devastating disease in a Sotheby’s auction benefiting the charity.
In the wake of Frederik’s diagnosis in 2016, the family founded The PolG Foundation, a United States Public Charity, to support and accelerate research to find effective treatments and a cure for PolG-related mitochondrial disorders. The Foundation brings together a wide range of experts in biotechnology and pharmaceuticals, as well as medical professionals and top universities, to study mitochondrial mutations and treat PolG-related disorders. Frederik serves as Creative Director.
“Once we got past that emergency phase, we realized that we had created this virtual community of research facilities, hospitals, laboratories, biotech companies, biotech funds and a good many doctors — the extraordinary medical community that has been supporting us over the last few years,” said Prince Robert. “With this community, we would be able to help others suffering from the same kind of challenges that we were dealing with and that Frederik was experiencing.”
In brief, mitochondria are membrane-bound organelles living inside our cells. They drive our body by helping move and convert various chemicals into energy. PolG is an enzyme that when mutated can rob the cells of energy, affecting organ function and overall bodily growth. Such mutations can also impact the nervous system, muscles and brain; often people who are diagnosed with MITO feel a constant state of energy depletion. To date, there are over two hundred mutations associated with MITO, including the Alpers-Huttenlocher syndrome — which causes recurrent and untreatable seizures, loss of movement, debilitating mental faculties and liver disease — and the Kearns-Sayre syndrome — which affects the muscles that control the eyes and eyelids.
As there is no known cure for PolG-related illnesses, The PolG Foundation has pledged to support innovative science and promising research in the field. Treatment today is often focused on supportive therapy, such as dietary management, vitamins and supplements, physical therapy and exercise, as well as the conservation or management of the body’s energy. The Foundation’s research aims to discover the origins of PolG-related diseases and explore new gene-therapy treatments. This research has broader implications for a host of other diseases and will potentially contribute to solving issues relating to Alzheimer’s, Parkinson’s, Diabetes, among many others.
The proceeds of Sotheby’s auction will benefit The PolG Foundation and help combat the disease by supporting such research. Prince Robert’s cellar is unlike any in the world, and the auction includes over four thousand individual bottles, including from top-tier Châteaux such as Haut-Brion, La Mission Haut-Brion, d’Yquem, Cheval Blanc, Lafite, Latour, Margaux, Mouton Rothschild, Lynch Bages, Palmer, Ausone and Petrus. Prince Robert stressed the bottles’ exceptional provenance, saying, “these bottles have really never left the cellars in Bordeaux.” The sale is extended through additions from Primum Familiae Vini (PFV), an international association of family-owned wineries, all of whom have donated very rare lots that have also never left their cellars.
Those wishing to donate directly can do so via The PolG Foundation.
