BCL-2 - PROLONGING LIFE IN A TRANSGENIC MOUSE MODEL OF FAMILIAL AMYOTROPHIC-LATERAL-SCLEROSIS

Mutations in the gene encoding copper/zinc superoxide dismutase enzyme produce an animal model of familial amyotrophic lateral sclerosis (FA LS), a fatal disorder characterized by paralysis. Overexpression of th e proto-oncogene bcl-2 delayed onset of motor neuron disease and prolo nged survival in transgenic mice expressing the FALS-linked mutation i n which glycine is substituted by alanine at position 93. it did not, however, alter the duration of the disease. Overexpression of bcl-2 al so attenuated the magnitude of spinal cord motor neuron degeneration i n the FALS-transgenic mice.