Chapter 9: Animal Models for ALS

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective motor neuron loss in the motor cortex and spinal cord, eventually leading to progressive paralysis and death. Despite many discoveries of ALS-related genes, the cause and pathogenesis of the disease remains to be elucidated. The motor neuron degeneration in ALS appears to be caused by multiple factors. To unravel a complex molecular basis of the disease, ALS animal models should be useful tools to monitor progression of the disease over the time and to determine crucial neurodegenerative events during the early disease course. To date, there are growing numbers of reports on ALS animal models. Most of them are rodent transgenic models overexpressing ALS-associated mutant genes, either constitutively or conditionally. In this chapter, we review recent advances in transgenic ALS animal models and discuss about their pathological features that may help our understanding of selective motor neuron degeneration in ALS.