Cyclosporin A Prolongs Survival of SOD1 Mutant Mice and Implicates Mitochondrial Permeability Transition in Amyotrophic Lateral Sclerosis

摘要: Amyotrophic lateral sclerosis (ALS) is a progressive upper and lower motor neuron degenerative disease characterized by loss of neurons in the spinal cord, brainstem, cortex (1, 2). It most common human disease, so far without cure with only limited treatment options. The combined both pyramidal tract degeneration creates picture weakness skeletal muscle wasting. Upper manifested histologically as corticospinal degeneration. Lower anterior horn nerve root atrophy. Brainstem nuclei cell usually spares oculomotor cranial nerves. Ascending leads to paralysis death 3– 5 yr after diagnosis from nerves that coordinate swallowing breathing (3). Sensation cognition are generally unaffected.