Friedreich’s Ataxia
Friedreich’s Ataxia: New Stem Cell Research
Stem cells are under investigation for their effectiveness in the treatment of neurologic disorders. Scientists have utilised the advantage of the cells especially mesenchymal stem cells (MSCs) to have the ability to secrete several factors via antioxidant effect. The factors secreted are helpful in the development of neurons. The use of stem cells in the therapeutic world has necessitated the importance in knowing their mechanism of action.
Researchers established a model of frataxin deficient cells through induction of reduced frataxin expression in a neuroblastoma cell line. The model was similar as is seen with Friedreich’s Ataxia due to the inadequate special kind of proteins .There was interruption in the antioxidant process henceforth no factors released for the protection of the neurons. The researchers went ahead, introduced the culture in a mesenchymal stem cell medium, and observed the progress. Increased levels of the frataxin proteins were noticed. There were also increased expressions of molecules susceptible to the anti-oxidative process.
The increase of an enzyme known as catalase due to introduction of the mesenchymal stem cells was beneficial because hydrogen peroxide scavenging is reported to be of great help in the deficiency of frataxin proteins as the number are now at increased levels. Effect of low frataxin proteins on other process such as cellular proliferation and differentiation was also analyzed. The researchers identified that a certain cell type, Schwann cells proved diminished formation and expansion. Schwann cells are responsible in providing a sheath on neuron axons. The Schwann cells of individuals suffering from Friedreich’s Ataxia are defective due to insufficient frataxin proteins.
Mesenchymal Stem Cells
The introduction of Mesenchymal Stem Cells had the advantage of differentiating Schwann cells in the period of research conducted by the scientists. Stem cell therapy
Mesenchymal Stem Cells secreted factors as explained shows protection against cellular dysregulation. The dysregulation is because of reduced frataxin proteins. The beneficial trait of this type of cells to secrete the factors essential in promoting frataxin proteins shows potential for its use in the management of Friedreich’s Ataxia. This disease has no absolute cure but the ongoing research is effective in reducing the processes involved in interfering with the function of the mitochondria. The researchers are positive on the outcome of the therapy in the management of this disease.
H/T: FriedreichsAtaxiaNews.com
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