A5001800992- Genetic Neurodegenerative Diseases
- Endoplasmic Reticulum Stress and Disease
- Mitochondrial Function and Pathology
- Metabolism, Diabetes, and Cancer
- Spaceflight effects on biology
- Heat shock proteins research
- Pancreatic function and diabetes
- Ion channel regulation and function
- Nicotinic Acetylcholine Receptors Study
- Genetic Mapping and Diversity in Plants and Animals
- Vagus Nerve Stimulation Research
Universidade do Porto
2018-2024
Rede de Química e Tecnologia
2018-2024
Stress response pathways like the integrated stress (ISR), mitochondrial unfolded protein (UPRmt) and heat shock (HSR) have emerged as part of pathophysiology neurodegenerative diseases, including Huntington's disease (HD) - a currently incurable caused by production mutant huntingtin (mut-Htt). Previous data from HD patients suggest that ISR is activated while UPRmt HSR are impaired in HD. The study these potential therapeutic targets requires cellular models mimic activation status found...
Acetylcholine (ACh) spillover from motor endplates occurs after neuronal firing bursts being potentiated by cholinesterase inhibitors (e.g., neostigmine). Nicotinic α7 receptors (α7nAChR) on perisynaptic Schwann cells (PSCs) can control ACh unknown mechanisms. We hypothesized that adenosine might be the gliotransmitter underlying PSCs-nerve terminal communication. Rat isolated hemidiaphragm preparations were used to measure (1) outflow of [3 H]ACh, (2) real-time transmitter exocytosis...
Abstract Developmental diseases are challenging to investigate due their clinical heterogeneity and relatively low prevalence. The Wolcott-Rallison Syndrome (WRS) is a rare developmental disease characterized by skeletal dysplasia permanent neonatal diabetes loss-of-function mutations in the endoplasmic reticulum stress kinase PERK (EIF2AK3). lack of efficient less invasive therapies for WRS highlights need new animal models that replicate complex pathological phenotypes, while preserving...
<h3>Background</h3> Huntington's disease (HD) is a protein misfolding disorder involving mutant form of the huntingtin (Htt) with polyglutamine expansion. Heat shock proteins (Hsp) are key therapeutic targets in diseases given their role folding, aggregation, and degradation. YM-1 an Hsp70 modulator (ATPase domain inhibitor) which was found protective SBMA. However, that consequences modulating specific Hsp seem to depend on nature client drug pharmacodynamics, it remains uncertain whether...
<h3>Background</h3> Huntington's disease (HD) is caused by mutant huntingtin (mHtt) and presents increased oxidative stress biomarkers, suggesting that aberrant redox signaling associated with HD pathogenesis. Treatment MitoQ, a mitochondria-targeted antioxidant, was neuroprotective in other models of neurodegenerative disorders, including ALS, Parkinson's Alzheimer's diseases. <h3>Aims</h3> Evaluate the efficacy MitoQ rescuing phenotypes vivo. <h3>Methods</h3> (500 µM) or vehicle were...