![]() ![]() In SMA-III mice long-term lithium administration determines a dramatic increase of survival motor neuron protein levels in the spinal cord. In addition, lithium prevents motor neuron enlargement and motor neuron heterotopy and suppresses the occurrence of radial-like glial fibrillary acidic protein immunostaining in the ventral white matter of SMA-III mice. Lithium administration attenuates the decrease in motor activity and provides full protection from motor neuron loss occurring in SMA-III mice, throughout the disease course. ![]() This model is characterized by very low levels of the survival motor neuron protein, slow disease progression and motor neuron loss, which enables to detect disease-modifying effects at delayed time intervals. In the present study we evaluated the long-term effects of lithium administration to a knock-out double transgenic mouse model (Smn-/- SMN1A2G+/- SMN2+/+) of Spinal Muscle Atrophy type III ( SMA-III). Protective effects of long-term lithium administration in a slowly progressive SMA mouse model.īiagioni, Francesca Ferrucci, Michela Ryskalin, Larisa Fulceri, Federica Lazzeri, Gloria Calierno, Maria Teresa Busceti, Carla L Ruffoli, Riccardo Fornai, Francesco ![]()
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