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The team looked at a protein called c-Jun, a transcription factor that regulates the expression of other genes. They found that the c-Jun protein plays a vital role in the regulating the plasticity of Schwann cells which is vital for the way in which the peripheral nervous system regenerates and repairs itself after injury.
Schwann cells produce the sheaths that surround and insulate neurons. When there is damage to the peripheral nervous system Schwann cells unwrap themselves from the degenerating axon. During this process of repair, Schwann cells then provide the correct environment for the neurons to re-grow and complete the process of repair.
By identifying this transcription factor, the research team believes that there is scope to produce eventual cures for damage and diseases of the peripheral nervous system, such as the inherited condition Charcot-Marie-Tooth disease and the autoimmune disorder Guillain-Barre disease.
Unlike the peripheral nervous system, the central nervous system does not regenerate when damaged. With further research, the team hopes to work towards identifying ways in which Schwann cells and c-Jun could be used to repair the spinal cord, leading to possible cures and relief for millions of people around the world suffering from damage of the central nervous system.
Further research could also identify whether abnormal activation of the c-Jun protein may be involved in causing Schwann cell tumours, for instance in the condition of neurofibromatosis type 2, leading to a better understanding of this condition and the development of therapies for this condition.
Dr. David Parkinson from the Peninsula Medical School, who was lead researcher on the paper, commented: "This is a very exciting first step towards understanding how the peripheral nervous system repairs itself, how that process could be used to produce cures for diseases of and damage to the peripheral nervous system, and how it could ultimately encourage the central nervous system to behave like the peripheral nervous system and repair itself."
He added: "We knew that Schwann cells, unlike other cells in the body, are constantly able to rejuvenate themselves. We now have a better understanding of how this happens, and that understanding could be used to create treatments and therapies for a wide range of degenerative diseases."
Adapted from materials provided by , via EurekAlert!, a service of AAAS.
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The life of Marie Schlau, a German Jewish girl born in 1833 hides great unsolved mysteries: accidents, disappearances, enigmas, unknown diagnoses, disturbing murders, love, tenderness, greed, lies, death ... alternatively a different story unfolds every time and takes us closer to the present. Thus, there are two parallel stories unravelling, each in a different age and place, which surprisingly converge in a revelatory chapter.
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Currently, BabelFAmily is financing two promising research projects aimed at finding a cure for Friedreich's Ataxia. Whenever you make a donation to us or purchase a copy of "The legacy of Marie Schlau", this is where all funds raised will be devoted to:
1) Gene Therapy for Friedreich's Ataxia research project:
The project is the result of an initiative of Spanish people affected by this rare disease who are grouped in GENEFA in collaboration with the Spanish Federation of Ataxias and the BabelFAmily. The Friedreich’s Ataxia Research Alliance (FARA), one of the main patients’ associations in the United States now joins the endeavour.
2) Frataxin delivery research project:
The associations of patients and families Babel Family and the Asociación Granadina de la Ataxia de Friedreich (ASOGAF) channel 80,000 euros of their donations (50% from each organisation) into a new 18-month project at the Institute for Research in Biomedicine (IRB Barcelona). The project specifically aims to complete a step necessary in order to move towards a future frataxin replacement therapy for the brain, where the reduction of this protein causes the most damage in patients with Friedreich’s Ataxia.
The study is headed by Ernest Giralt, head of the Peptides and Proteins Lab, who has many years of experience and is a recognised expert in peptide chemistry and new systems of through which to delivery drugs to the brain, such as peptide shuttles—molecules that have the capacity to carry the drug across the barrier that surrounds and protects the brain. Since the lab started its relation with these patients’ associations in 2013*, it has been developing another two projects into Friedrich’s Ataxia.