These data demonstrate that long-term lithium administration during a long-lasting motor neuron disorder attenuates behavioural deficit and neuropathology. 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
