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Spanish scientists generate neurons affected by degenerative processes
14 June 2017 @ 11:42

      Today, I would like to show you an important news for Medicine: “Scientists manage to generate neurons affected by neurodegenerative processes”.

      Effectively, researchers from the Institute of Bioengineering of Catalonia (IBEC) have managed to generate neurons affected by two different neurodegenerative processes, from the skin cells of a patient with a rare neurodegenerative disease, the Gerstmann-Sträussler-Scheinker syndrome.

      According to the researchers, the ability to develop cellular models with characteristics of two unrelated neurodegenerative diseases, in the same neurons, will help in the study of other neurodegenerative diseases, with a similar diagnosis, such as Alzheimer's.

      The study, published in the journal “Molecular Neurobiology”, was a collaboration between IBEC researchers, led by José Antonio del Río, also a professor at the University of Barcelona (UB); Adolfo López de Munain, neurologist of the Hospital of Donostia; and Isidre Ferrer, of the Pathology Department of Bellvitge Hospital, all members of the Center for Biomedical Research in the Network of Neurodegenerative Diseases (CIBERNED).

Jose Antonio del Rio, on left side, and his team

      The research began when doctors, who analysed the brain of a deceased patient, found that, in addition to tauopathy  --a neurodegenerative disorder— it also had a genetic mutation, that causes a much more rare neurodegenerative disease, the Gerstmann-Sträussler-Scheinker syndrome (GSS ), a prionopathy involving a mutation of the PRNP prion protein gene.

      According to the IBEC researcher, Andreu Matamoros, finding two neurodegenerative diseases, with a different origin,  --a tauopathy and a prionopathy--,  in the same patient, aroused the curiosity of the doctors, which led them to contact the IBEC researchers.

A human brain

      "This unexpected relationship opens the door to the search for new therapeutic targets that prevent both diseases from complementing each other to provoke degeneration and neuronal death", Matamoros, the study's lead author, has said.

      The researchers had the support of a member of the family of the patient, carrying the same mutation, who donated cells from his skin. They reprogramed these somatic cells and converted them into neurons, to study the relationship between tauopathy and the mutation observed in the PRNP gene. "The neurons, generated in this study, are the first cell line induced from fibroblasts of a GSS patient", Jose Antonio Del Rio, research coordinator, has said.

      To reprogram the fibroblasts, to generate neurons, they were first reprogrammed into induced pluripotent stem cells, a transition developed by the Angel Raya group of the Center for Regenerative Medicine in Barcelona.

Angel raya in the laboratory

      The second step, which consisted in differentiating iPS cells into neurons, was carried out, in parallel, in the groups of Rosario Sánchez-Pernaute (Inbiomed), Joaquín Castilla (CiCBiogune) and José Antonio Del Río (IBEC).

Rosario Sanchez-Pernaute in a laboratory of Imbiomed

       "We work in parallel because of the complexity of creating neurons from pluripotent cells. Fortunately, we found that the two proposed methods successfully generate fully functional neurons", Del Río has said.

      Well, I hope this news has seemed to you as interesting as to me.

      Until my next post, kind regards,


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