Publications by authors named "Santiago Rico-Rios"
Article Synopsis
- - Amyotrophic lateral sclerosis (ALS) is a severe motor neuron disease with about a three-year average survival time, primarily characterized by TDP-43 protein issues that affect gene stability and autophagy processes.
- - Research on ALS mice revealed that reducing ATG4B worsens survival and autophagy, while an increase in LC3ylation was observed in both ALS patients and mouse models, suggesting a link between these processes.
- - Antisense oligonucleotides (ASOs) targeting TDP-43 genes have been developed, showing potential for non-invasive treatments that can effectively distribute in the brain after administration.
The dysfunction of TAR DNA-binding protein 43 (TDP-43) is implicated in various neurodegenerative diseases, though the specific contributions of its toxic gain-of-function versus loss-of-function effects remain unclear. This study investigates the impact of TARDBP loss on cellular metabolism and viability using human-induced pluripotent stem cell-derived motor neurons and HeLa cells. TARDBP silencing led to reduced metabolic activity and cell growth, accompanied by neurite degeneration and decreased oxygen consumption rates in both cell types.
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