Protein that Repaired Lung Fibrosis in Mice is All the RAGE
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A team of researchers discovered that a protein called RAGE can fix damage inflicted to DNA molecules, and also repair lung scarring in mice.
The study reporting the findings, “Homeostatic nuclear RAGE-ATM interaction is essential for efficient DNA repair,” was published in the journal Nucleic Acids Research.
The RAGE protein, short for “receptor of advanced glycation endproducts,” is found mainly at the surface of cells where it works as a receptor capable of binding to different molecules (ligands). Dysregulation of RAGE and its ligands are known to contribute to the development of several disorders, including diabetes, Alzheimer’s disease and cancer.
Now, researchers at the Heidelberg University Hospital and the German Center for Diabetes Research discovered that the protein also can be found inside cells, in the nucleus, where DNA is stored. Here, the protein was found to be responsible for the repair of severe damages in DNA, those called double-strand breaks.
This type of damage leads to a complete break in both DNA strands that, if left unrepaired, results in cell death.
Damage to DNA is relatively frequent in our cells. Therefore, cells have key repair mechanisms, but upon their failure the conditions are set for diseases, such as aging, degeneration and scarring.
Researchers found that mice lacking RAGE due to a genetic defect developed pulmonary fibrosis. Lungs are in constant contact with the “outside world,” and are thus particularly prone to factors that induce damage. As a consequence, they require active mechanisms of DNA repair.
When the team reintroduced RAGE into the mice’s lungs, they not only rescued the lungs’ DNA repair mechanisms, but also the scarring (fibrosis); the lungs’ scar tissue regenerated and re-acquired part of its function.
“This is astonishing in that fibrosis has so far been considered irreversible. With RAGE, we could for the first time have found a possible starting point to cure this frequent tissue damage,” Peter Nawroth, MD, medical director of the Department of Endocrinology, Metabolism and Clinical Chemistry at Heidelberg University Hospital, and the study’s lead author, said in a press release.
“Many questions – e.g. how this healing works in detail – are still unanswered” Nawroth added.
Varum Kumar, the study’s first author, concluded “For the first time ever, molecular therapy for the repair of damage to the lungs caused by genes and cells and thus for the prevention of fibrosis or tumors, which also occur as a result of DNA damage, may be within reach.”
Now researchers aim to investigate the role of the RAGE protein in fibrosis of other organs, namely the liver and kidney, and unveil its potential therapeutic use in fibrosis.
Larry
Any trials underway for those with IPF?
Barry Gauthier
When will this turn into a treatment for human use?
Barry Gauthier
Looking for other uses is fine. But if you already know that it works on fibrosis, why not try to get it fast tracked so youu can start saving lives. Every day, people are dying from fibrosis.
Cristy
I was thinking the same thing. Let’s get moving on this so more people have a longer and better quality of life. It’s like teasing a cupcake lover with a giant cupcake they can’t eat!!
Barbara Palmer
We need every possibility that might help us. We need this drug fast tracked. What have we got to lose but time? I would so do it.
Bonnie Miller
I was on Esbriet for several months then had a reaction and was switched to Ofev. I truly believe between these meds and my faith in God that they have lengthened my life. I have UIP which is a rare form of pulmonary fibrosis. I was diagnosed in November 2016 and my doctor didn't give much hope past two years but I am going strong.....thank you Jesus!
Roger Rowe
When will it be ready to treat PF in people.
Mary Lasowski
Yeppie hope it works, soon.
Trevor Hayes
Is'nt this the conventional medical equivalent to what the natural supplement Serrepeptase has been doing naturally for a few decades already?
Betty Atkinson
hope it soon
Barbara Palmer
I have IPF I totally would do trial.
EduardoACasiano
I volunteer to by a guinea pig for your trials.
Mary Nuzzi
Hope they come up with something soon. Hate pulling this heavy POC around and having SOB upon exertion. my path is getting shorter.
John Aldridge
What's the deal? They need to get this started. I go to Emory next week. Is this going to be one of the things that will save my life? I would be willing to do it. If this would save lives then they need to get it going.
Stan L'Hoste
Blogs are needed so patients can exchange ideas, support, grief, experience. I have been trying for close to 2 yrs now to find someone who has progressed further than myself and what they did to prepare for limited life. Doctors are not very straight forward and have no resources when it comes to psychological, emotional or family needs and preparation. There definitely needs to be a key registration that houses all patient history and queries all clinical trials being done to notify patients of matching criteria and notifies them for applying to trials at the same time it advises the party conducting the trial.. Communications is terrible and everyone I talk with knows it, but nothing exist, so they really need to build a better mousetrap!!!!!! I would do anything possible to prevent someone going thru what I have, [email protected]
Barry Gauthier
I totally agree.
Barry McGee
You are absolutely right. I've found a support sit called TeamInspire.com. Try the Living With IPF section, the people there are great. Also, I understand that University of North Carolina is doing stem cell studies. Also find a Center of Excellence hospital with an ILD team and go there.
John Williams
I have IPF and found this interesting
Vinoth
Please release the drugs for lung ASAP. My dad is suffering a lot.Vinoth from India
DRodman
My mother passed away in August of 2017, due to (most likely drug-induced) pneumonitis and IPF. She had fought breast cancer, and we believe the cancer drugs, combination of cancer drugs and radiation, or even possibly the post-chemo bone density drug was the culprit.
She was also an asthmatic, and mistook her symptoms for an attack. She drove herself to the ER, was admitted to the ICU, and never went home.
I wish these cutting-edge treatment were available to patients who have no other options. My mom was simply treated with massive doses of steroids, which had no effect at all.
Richard J Shank
Curious if there is any possibility of volunteering for testing of RAGE.
Roger Rowe
This was posted almost a year ago, what is the latest info on this ?
Barbara Palmer
We NEED this NOW. Please give update. I would be happy to be a RAT!
Michael
Any recent news? My Dad is suffering from Pulminary Fibrosis,a terrible disease...
StephanieGarmon-Ritchie
My son in law has this now. 10 yrs ago he had his larynx removed due to cancer. Found cancer in thyroid. Did 40 radiation treatments, now this. Is their anything to take that is a source of RAGE? ANY INFO WOULD BE SO APPRECIATED.
Ann Hubbard
Why are there no responses to questions about a trial or where the research is today?