Modelli murini di disfunzione midollare spinale basati sulla degenerazione transgenica o sulla rimozione neurotossica di popolazioni motoneuronali. Caratterizzazione e approcci preliminari di terapia cellulare

In this study we have identified two models of motor neuronal degeneration in which we attempted, in a second phase, a preliminary approach of cell transplantation. First of all, we characterized from a pathological and functional point of view the G93A mouse, a transgenic animal model that develops a disease very similar to human amyotrophic lateral sclerosis, highlighting how the number and volume of motor neurons and the density of cholinergic processes are correlated one another and with the functional progressively deteriorating conditions of the animals. We transplanted, in the spinal cords of these mice, immortalized human precursors, but in none of the cases implanted cells survived, probably due to an improper immunosopressive protocol. In the second model, we induced a lesion in healthy rats by the intramuscular injection of Volkensin, a neurotoxin that produces selective motor neuronal degeneration. After assessing the goodness of the model, we performed transplantation, in newborn and adult animals, with immortalized human precursors and with rat neuroblasts. In both cases, we observed how implanted cells survived and integrated in the host tissue, although in the case of human precursors we could not identify to which cellular line they differentiated.