**1. Introduction**

174 Amyotrophic Lateral Sclerosis

Pieri M, Albo F, Gaetti C, Spalloni A, Bengtson CP, Longone P, Cavalcanti S, Zona C (2003)

Pun S, Santos AF, Saxena S, Xu L, Caroni P (2006) Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF. 9:408-419. Quinlan KA, Schuster JE, Fu R, Siddique T, Heckman CJ (2011) Altered postnatal maturation

Sasaki S, Maruyama S (1992) Increase in diameter of the axonal initial segment is an early

Schwindt PC, Crill WE (1977) A persistent negative resistance in cat lumbar motoneurons.

Sobue G, Matsuoka Y, Mukai E, Takayanagi T, Sobue I, Hashizume Y (1981) Spinal and

Stephens B, Guiloff RJ, Navarrete R, Newman P, Nikhar N, Lewis P (2006) Widespread loss

Sunico CR, Dominguez G, Garcia-Verdugo JM, Osta R, Montero F, Moreno-Lopez B (2011)

Takahashi H, Oyanagi K, Ikuta F (1993) The intermediolateral nucleus in sporadic

Turner BJ, Talbot K (2008) Transgenics, toxicity and therapeutics in rodent models of mutant

van Zundert B, Peuscher MH, Hynynen M, Chen A, Neve RL, Brown RH, Jr., Constantine-

Williams C, Kozlowski MA, Hinton DR, Miller CA (1990) Degeneration of spinocerebellar

Zona C, Pieri M, Carunchio I (2006) Voltage-Dependent Sodium Channels in Spinal Cord

neurons in amyotrophic lateral sclerosis. Ann Neurol 27:215-225.

of Amyotrophic Lateral Sclerosis. J Neurophysiol 96:3314-3322.

Paton M, Bellingham MC (2008) Neonatal Neuronal Circuitry Shows Hyperexcitable Disturbance in a Mouse Model of the Adult-Onset Neurodegenerative Disease Amyotrophic Lateral Sclerosis. J Neurosci 28:10864-

Motor Neurons Display Rapid Recovery From Fast Inactivation in a Mouse Model

change in amyotrophic lateral sclerosis. J Neurol Sci 110:114-120.

amyotrophic lateral sclerosis. Neurosci Lett 351:153-156.

disease. A morphometric study. J Neurol Sci 244:41-58.

amyotrophic lateral sclerosis. Acta Neuropathol 86:190-192.

SOD1-mediated familial ALS. Prog Neurobiol 85:94-134.

589:2245-2260.

Brain Res 120:173-178.

Neuropathol 55:227-235.

Neurol Sci 22:1-24.

10874.

Altered excitability of motor neurons in a transgenic mouse model of familial

of electrical properties in spinal motoneurons in an ALS mouse model. J Physiol

cranial motor nerve roots in amyotrophic lateral sclerosis and X-linked recessive bulbospinal muscular atrophy: morphometric and teased-fiber study. Acta

of neuronal populations in the spinal ventral horn in sporadic motor neuron

Reduction in the motoneuron inhibitory/excitatory synaptic ratio in an earlysymptomatic mouse model of amyotrophic lateral sclerosis. Brain Pathol 21:1-15. Swash M, Fox KP (1974) The pathology of the human muscle spindle: effect of denervation. J Amyotrophic lateral sclerosis (ALS) is a devastating, fast progressing and fatal disease for which there is little treatment. It is marked by loss of spinal and cortical motoneuron function. Many parameters are altered in the time leading up to this loss, including electrical properties, endoplasmic reticulum (ER) stress, glial functioning, glutamate signaling, protein degradation, mitochondrial functioning, axonal transport, and immune response. This chapter concentrates on the interplay between altered electrophysiological properties and molecular events. Emphasis is placed on the changes that precede overt symptom onset and results are mainly drawn from studies using the rodent models of ALS.
