Bcl2a1 serves as a switch in death of motor neurons in amyotrophic lateral sclerosis

Dear Editor,

Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease, characterised by the progressive degeneration of motor neurons.¹ Although most of the ALS cases are sporadic, about 10% of the cases are familial (fALS). Among fALS cases, one-fifth are linked to mutations in the gene encoding Cu, Zn superoxide dismutase (SOD1). The mechanisms through which mutations in SOD1 lead to late-onset motor neuron degeneration are not fully understood, even though it is established that mutant SOD1s acquire novel or enhanced neurotoxic properties.² It is now well accepted that, even when due to a single gene defect, ALS is a multi-systemic disease resulting from a complex neurotoxic cascade that involves a crosstalk between motor neurons and glia and between motor neurons and muscle.^{1, 3, 4, 5} Previous studies indicate that reactive oxygen species, nitric oxide, cytokines and growth factors are among the players involved in a cascade of events leading to apoptotic death of motor neurons.^{1, 6}

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