Amyotrophic lateral sclerosis: a focus on disease progression

Abstract

Since amyotrophic lateral sclerosis (ALS) was discovered and described in 1869 as a neurodegenerative disease in which motor neuron death is induced, a wide range of biomarkers have been selected to identify therapeutic targets. ALS shares altered molecular pathways with other neurodegenerative diseases, such as Alzheimer's, Huntington's, and Parkinson's diseases. However, the molecular targets that directly influence its aggressive nature remain unknown. What is the first link in the neurodegenerative chain of ALS that makes this disease so peculiar? In this review, we will discuss the progression of the disease from the viewpoint of the potential biomarkers described to date in human and animal model samples. Finally, we will consider potential therapeutic strategies for ALS treatment and future, innovative perspectives.

Figure 1

Schematic representation of the different molecular pathways altered in amyotrophic lateral sclerosis. Although the trigger for neurodegeneration remains unknown, all of these deregulated mechanisms prompt motor neuron death.

Figure 2

Time course of disease progression and identification of related potential biomarkers involved in each stage. Reliable biomarkers can be helpful in the prediction of different disease stages and will facilitate the application of accurate therapeutic treatments.