Posts Tagged ‘fratagene’

This morning I read a really scary and exciting story. A true story!

It’s about the newborn baby Frataxin who is destined to play an important role in keeping the nerve system safe when they grow up. To become an adult they have to travel the dark and dangerous roads to the safe city of Mitochondria. The distant lights from Mitochondria draws them closer and closer on their way to become the true heroes they are meant to be. In the darkness the Inspectors of Death are luring. Their mission is to watch the baby Frataxins and to tag them with Ubiquitin dust, the Kiss of Death. The Frataxin babies unlucky to have been tagged is immediately attacked by the Death Squad and transported  to the Hall of Proteasome where they are melted in the great fire. They will never become the Protectors of the nerves as they were meant to.


Until one day when the superheroes arrive. With their great powers they throw a protective shield around all the Frataxin babies and knocks out the Inspectors of Death. The superheroes follow the Frataxin babies all the way from the crib and to the city of Mitochondria where they grow up to fulfill their destiny to protect and defend the nerve cells.


The end.


Hahaha so much drama! Well, this is what I pictured in my head when I was reading this research report  from Team Testi this morning:

E3 Ligase RNF126 Directly Ubiquitinates Frataxin, Promoting Its Degradation: Identification of a Potential Therapeutic Target for Friedreich Ataxia.




“The kiss of death” is actually a term used in medicine for the process when the protein is tagged before destruction. The ones who discovered this process actually won a Nobel price for it.


When you have a really dangerous and evil beast, like FA, you have to target it from all angles to kill it. What these researchers have discovered is the substance in charge of destructing the frataxin protein. This is a completely normal and necessary process that constantly goes on. New proteins are created from our gene codes and later they are broken down. It happens in all creatures.


The thing for FA patients is that our cells produce so little frataxin already. We really want to keep it all, right?


These researchers have found that E3 Ligase RNF123 plays an important role in the natural destruction of frataxin. Now that it is identified they can start finding treatments that aim to stop this process. (The treatment = the superheroes from my little story.) So while they look for treatments that increase the frataxin production they can at the same time stop the frataxin from being destructed. More frataxin for me and more frataxin for you. I love it!


There is so much exciting research going on at the moment. The fact that so many brilliant people dedicate their time, brain and life to find a cure for you and me is so amazing. THANK YOU! ❤


As you know I really have a weak spot for Team Testi as they gave me my life back through interferon gamma. “Team Testi” is my own name. The company name is actually Fratagene. Their slogan is: “One Disease, One Commitment” Check out their web page here.
This picture is borrowed from FARA @curefa_org on instagram. Go follow them 🙂

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