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Histone deacetylases and their role in motor neuron degeneration
TLDR
The present evidence of transcriptional dysregulation in ALS, the role of histone deacetylases (HDACs) in disease pathogenesis, and the novel pharmacologic strategies that are being comprehensively studied to prevent motor neuron death are discussed, with focus on sirtuins (SIRT) and their effectors. Expand
Trophic factors as modulators of motor neuron physiology and survival: implications for ALS therapy
TLDR
Experimental data is summarized on the role of trophic factors in motor neuron function and survival, as well as their mechanisms of action. Expand
Chronic infusion of SOD1G93A astrocyte‐secreted factors induces spinal motoneuron degeneration and neuromuscular dysfunction in healthy rats
TLDR
It is demonstrated that factors released in vitro from astrocytes derived from ALS mice cause spinal motoneuron death and consequent neuromuscular dysfunction in vivo. Expand
Cyclic GMP-AMP synthase promotes the inflammatory and autophagy responses in Huntington disease
TLDR
It is reported that cGAS, a DNA damage sensor, is highly upregulated in the striatum of a mouse model and HD human patient’s tissue and is a critical regulator of inflammatory and autophagy responses in HD. Expand
RasGRP1 is a causal factor in the development of l-DOPA–induced dyskinesia in Parkinson’s disease
TLDR
It is demonstrated that RasGRP1 [(guanine nucleotide exchange factor (GEF))] controls the development of LID, a critical striatal regulator of Parkinson's disease treatment. Expand
Spinal inhibitory circuits and their role in motor neuron degeneration
TLDR
In conclusion, inhibitory failure by either mechanism might lead to motor neuron degeneration, and this suggests inhibitory circuits and Renshaw cells as pharmacologic targets for ALS treatment. Expand
Excitatory and Inhibitory Neuronal Circuits in the Spinal Cord and Their Role in the Control of Motor Neuron Function and Degeneration.
TLDR
These studies have shown that the coordinated activity of several interneuron types, mainly GABAergic and glycinergic inhibitory neurons, have a crucial role in the modulation of motor neurons activity that finally excites the corresponding muscles. Expand
Neuropathological characterization of spinal motor neuron degeneration processes induced by acute and chronic excitotoxic stimulus in vivo
TLDR
It is concluded that acute AMPA-induced excitotoxicity induces MN loss by necrosis, while the progress of degeneration induced by chronic infusion is slow, starting with an early apoptotic process followed by necrotic process. Expand
Role of Mitochondrial Dysfunction in Motor Neuron Degeneration in ALS
Amyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper andExpand
Chronic GABAergic blockade in the spinal cord in vivo induces motor alterations and neurodegeneration
TLDR
It is demonstrated that the chronic infusion of bicuculline enhanced the excitotoxic effect of AMPA, causing faster bilateral paralysis and increasing motor neuron loss, suggesting that their alterations may be involved in the neurodegeneration processes characteristic of motor neuron diseases such as amyotrophic lateral sclerosis. Expand
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