Abstract: Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease affecting motor neurons
(MN). This fatal disease is characterized by progressive muscular atrophy and unfortunately it does not have
an effective treatment. Although a small proportion of ALS cases have a familiar origin, the vast majority of
them are thought to have a sporadic origin. Although the pathogenesis of ALS has not been fully elucidated, various
disorders in different cellular functions such as gene expression, protein metabolism, axonal transport and
glial cell disorders have been linked to MN degeneration. Among them, proteostasis is one of the best studied.
Retinoids are vitamin A-derived substances that play a crucial role in embryogenesis, development, programmed
cell death and other cellular functions. Retinoid agonists behave as transcription factors throughout the activation
of the nuclear retinoid receptors.
Several reports in the literature suggest that retinoids are involved in proteostasis regulation, by modulating its
two major pathways, the ubiquitin-proteasome system and the autophagy-lysosome response. Additionally,
there are some evidences for a role of retinoids themselves, in ALS pathogenesis. In this review, we discuss the
importance of proteostasis disruption as a trigger forMNdegeneration and the capability of retinoids tomodulate
it, as well as the potential therapeutic role of retinoids as a new therapy in ALS.
Otras publicaciones de la misma revista o congreso con autores/as de la Universidad de Cantabria