The Role of Pericytes in Amyotrophic Lateral Sclerosis

Adv Exp Med Biol. 2019:1147:137-146. doi: 10.1007/978-3-030-16908-4_6.

Abstract

In amyotrophic lateral sclerosis (ALS), motor neurons die selectively. Therefore, initial symptoms that include fasciculation, spasticity, muscle atrophy, and weakness emerge following axons retraction and consequent muscles' denervation. Patients lose the ability to talk and swallow and rely on parenteral nutrition and assisted ventilation to survive. The degeneration caused by ALS is progressive and irreversible. In addition to the autonomous mechanism of neuronal cell death, non-autonomous mechanisms have been proved to be toxic for motor neurons, such as the activation of astrocytes and microglia. Among the cells being studied to unveil these toxic mechanisms are pericytes, cells that help keep the integrity of the blood-brain barrier and blood-spinal cord barrier. In this chapter, we aim to discuss the role of pericytes in ALS.

Keywords: Amyotrophic lateral sclerosis; Blood–brain barrier; Blood–spinal barrier; Lou Gehrig’s disease; Microhemorrhage; Motor neuron; Pericyte; Pericyte loss; Perivascular cells; SOD1 mice.

MeSH terms

  • Amyotrophic Lateral Sclerosis*
  • Animals
  • Axons
  • Disease Models, Animal
  • Humans
  • Mice, Transgenic
  • Motor Neurons
  • Pericytes*
  • Superoxide Dismutase

Substances

  • Superoxide Dismutase