Motor neuron diseases (MNDs) are a rather heterogeneous group of diseases, with either sporadic or genetic origin or both, all\r\ncharacterized by the progressive degeneration of motor neurons. At the cellular level, MNDs share features such as protein misfolding\r\nand aggregation, mitochondrial damage and energy deficit, and excitotoxicity and calcium mishandling. This is particularly\r\nwell demonstrated in ALS, where both sporadic and familial forms share the same symptoms and pathological phenotype, with\r\na prominent role for mitochondrial damage and resulting oxidative stress. Based on recent data, however, altered control of gene\r\nexpression seems to be a most relevant, and previously overlooked, player in MNDs. Here we discuss which may be the links that\r\nmake pathways apparently as different as altered gene expression, mitochondrial damage, and oxidative stress converge to generate\r\na similar motoneuron-toxic phenotype.
Loading....