Pier Lorenzo Puri, M.D PhD Professor in the Development, Aging and Regeneration Program Sanford Burnham Prebys Medical Discovery Institute

Maybe Spinal Repair Cells Can Be Turned On and Off

MedicalResearch.com Interview with:

Pier Lorenzo Puri, M.D PhD Professor in the Development, Aging and Regeneration Program Sanford Burnham Prebys Medical Discovery Institute

Dr. Puri

Pier Lorenzo Puri, M.D PhD
Professor in the Development, Aging and Regeneration Program
Sanford Burnham Prebys Medical Discovery Institute

MedicalResearch.com: What is the background for this study? What are the main findings?

Response: My lab has been studying special repair cells called fibro-adipogenic progenitors (FAPs) and how these cells change in models of motor neuron diseases. These cells usually repair muscles after acute injury. But we are finding the FAPs change dramatically in disease settings.

In this study we looked at these cells in models of spinal cord injury, ALS and spinal muscular atrophy, including muscle tissue from ALS patients. We found that FAPs change radically in several ways.

Most importantly, the cells used a different signaling pathway, IL-6-STAT3, and when we blocked this signaling muscle atrophy and fibrosis halted. While further studies in humans are needed, this is a promising finding as FDA-approved medicines that block IL-6 and STAT3 are available.

MedicalResearch.com: What should readers take away from your report?

Response: In models of motor neuron diseases, FAP cells change radically. They accumulate inside muscles and also turn on a different signaling path, which can be targeted to halt disease symptoms. While very early, these findings are promising as FDA-approved drugs that inhibit this signaling pathway exist. These cells could also be used as a prognostic marker for motor neuron diseases.

MedicalResearch.com: What recommendations do you have for future research as a result of this work?

Response: We are eager to study how we could selectively remove these disease-specific FAPs or convert the cells so they can repair nerves.

More studies could be done to correlate the identity of the FAPs with disease progression in humans. This would validate the cells as potential prognostic markers for motor neuron diseases.

MedicalResearch.com: Is there anything else you would like to add?

Response: We previously studied these same FAPs in models of muscular dystrophy and found they changed in this same manner. This adds evidence that FAPs are drivers of motor neuron disease symptoms.

Citation:

Luca Madaro, Magda Passafaro, David Sala, Usue Etxaniz, Francesca Lugarini, Daisy Proietti, Maria Vittoria Alfonsi, Chiara Nicoletti, Sole Gatto, Marco De Bardi, Ricardo Rojas-García, Lorenzo Giordani, Sara Marinelli, Vittoria Pagliarini, Claudio Sette, Alessandra Sacco, Pier Lorenzo Puri. Denervation-activated STAT3–IL-6 signalling in fibro-adipogenic progenitors promotes myofibres atrophy and fibrosis. Nature Cell Biology, 2018; 20 (8): 917 DOI: 10.1038/s41556-018-0151-y

Aug 21, 2018 @ 5:27 pm

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