A5011748125- Genetic Neurodegenerative Diseases
- Muscle Physiology and Disorders
- Neurogenetic and Muscular Disorders Research
- Pain Mechanisms and Treatments
- Neuroscience and Neuropharmacology Research
- Neuropeptides and Animal Physiology
- Neurotransmitter Receptor Influence on Behavior
- Protein Hydrolysis and Bioactive Peptides
- Pharmacological Receptor Mechanisms and Effects
- Ion channel regulation and function
- Marine Toxins and Detection Methods
- Cancer-related gene regulation
- Animal testing and alternatives
- Nicotinic Acetylcholine Receptors Study
- Receptor Mechanisms and Signaling
- Insect Utilization and Effects
- Signaling Pathways in Disease
- Genetics and Neurodevelopmental Disorders
- Endoplasmic Reticulum Stress and Disease
- Nuclear Structure and Function
- Enzyme function and inhibition
- Insect Resistance and Genetics
- Neuroscience of respiration and sleep
- Neuroendocrine regulation and behavior
- Regulation of Appetite and Obesity
Florida State University
2022-2024
Dulbecco Telethon Institute
2014-2023
University of Trento
2014-2023
Universidad San Pablo CEU
2008-2021
Italian Institute of Technology
2014-2016
Universidad de Valladolid
1990-1992
Polyglutamine expansion in androgen receptor (AR) is responsible for spinobulbar muscular atrophy (SBMA) that leads to selective loss of lower motor neurons. Using SBMA as a model, we explored the relationship between protein structure/function and neurodegeneration polyglutamine diseases. We show here arginine methyltransferase 6 (PRMT6) specific co-activator normal mutant AR interaction PRMT6 with significantly enhanced mutant. occurs through steroid motif, LXXLL, activating function 2...
Abstract Spinal and bulbar muscular atrophy (SBMA) is characterized by loss of motoneurons sensory neurons, accompanied muscle cells. SBMA due to an androgen receptor containing a polyglutamine tract (ARpolyQ) that misfolds aggregates, thereby perturbing the protein quality control (PQC) system. Using AR113Q mice we analyzed proteotoxic stress-induced alterations HSPB8-mediated PQC machinery promoting clearance misfolded proteins autophagy. In symptomatic male mice, found expression...
Spinobulbar muscular atrophy (SBMA) is an X-linked neuromuscular disease caused by polyglutamine (polyQ) expansion in the androgen receptor (AR) gene. SBMA belongs to family of polyQ diseases, which are fatal neurodegenerative disorders mainly protein-mediated toxic gain-of-function mechanisms and characterized deposition misfolded proteins form aggregates. The neurotoxicity can be modified phosphorylation at specific sites, thereby providing rationale for development disease-specific...
Spinocerebellar ataxia type 35 (SCA35) is a rare autosomal-dominant neurodegenerative disease caused by mutations in the TGM6 gene, which codes for transglutaminase 6 (TG6). Mutations TG6 induce cerebellar degeneration an unknown mechanism. We identified seven patients bearing new TGM6. To gain insights into molecular basis of mutant TG6-induced neurotoxicity, we analyzed all mutants and five previously linked to SCA35. found that wild-type (TG6-WT) protein mainly localized nucleus...
Polyglutamine (polyQ) expansions in the androgen receptor (AR) gene cause spinal and bulbar muscular atrophy (SBMA), a neuromuscular disease characterized by lower motor neuron (MN) loss skeletal muscle atrophy, with an unknown mechanism. We generated new mouse models of SBMA for constitutive inducible expression mutant AR performed biochemical, histological functional analyses phenotype. show that polyQ-expanded causes dysfunction, premature death, IIb-to-IIa/IIx fiber-type change,...
ABSTRACT Pleiotrophin (PTN), a neurotrophic factor with important roles in survival and differentiation of dopaminergic neurons, is up‐regulated the nucleus accumbens after amphetamine administration suggesting that PTN could modulate amphetamine‐induced pharmacological or neuroadaptative effects. To test this hypothesis, we have studied effects genetically deficient (PTN −/−) wild type (WT, +/+) mice. In conditioning studies, found induces conditioned place preference both −/− WT (+/+) When...
Abstract Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease characterized by the loss of lower motor neurons. SBMA caused expansions polyglutamine tract in gene coding for androgen receptor (AR). Expression polyglutamine-expanded AR causes damage to neurons skeletal muscle cells. Here we investigated effect β-agonist stimulation myotube cells derived from mice patients, knock-in mice. We show that treatment myotubes expressing with clenbuterol increases their size....
Spinal and bulbar muscular atrophy is caused by polyglutamine (polyQ) expansions in androgen receptor (AR), generating gain-of-function toxicity that may involve phosphorylation. Using cellular animal models, we investigated what kinases phosphatases target polyQ-expanded AR, whether polyQ modify AR phosphorylation, how this contributes to neurodegeneration. Mass spectrometry showed preserve native phosphorylation increase at conserved sites controlling stability transactivation. In...
Stress-induced behaviours are driven by complex neural circuits and some neuronal populations concurrently modulate diverse behavioural physiological responses to stress. Glucagon-like peptide-1 (GLP-1)-producing preproglucagon (PPG) neurons within the lower brainstem caudal nucleus of solitary tract (cNTS) particularly sensitive stressful stimuli implicated in multiple interoceptive psychogenic threats. However, afferent inputs driving stress-induced activation PPG largely unknown, role...
ABSTRACT Short‐term incubation with pharmacologically relevant concentrations of morphine has been shown to transiently affect the metabolism and redox status NG108‐15 cells through δ‐opioid receptor stimulation, but apparently did not provoke cell death. The present work tries determine if at longer time intervals (24 h) provokes apoptosis and/or necrosis, as it described in other lines. We have also checked potential modulatory role yohimbine on these effects, basis previously interactions...
Abstract Stress-induced behaviours are driven by complex neural circuits and some neuronal populations concurrently modulate diverse behavioural physiological responses to stress. Glucagon-like peptide-1 (GLP1)-producing preproglucagon (PPG) neurons within the lower brainstem caudal nucleus of solitary tract (cNTS) particularly sensitive stressful stimuli implicated in multiple interoceptive psychogenic threats. However, afferent inputs driving stress-induced activation PPG largely unknown,...
Food-related disorders are increasingly common in developed societies, and the psychological component of these has been gaining increasing attention. Both overnourishment with high-fat diets perinatal undernourishment mice have linked to a higher motivation toward food, resulting an alteration food intake. Clusterin (CLU), multifaced protein, is overexpressed nucleus accumbens (NAc) overfed rats, as well those that suffered chronic undernutrition. Moreover, increase this protein was...
ABSTRACT Angiotensin I(AI)-converting enzyme (ACE) (EC 3.4.15.1) was solubilized from the membrane fraction of chicken lung using trypsin and nonidet P40 extraction, then purified to homogeneity by captopril affinity chromatography. Comparison trypsin-extracted detergent-solubilized membrane-bound converting sodium dodecyl sulphatepolyacrylamide gel electrophoresis isoelectric focusing indicated that membrane-binding sequence contributed a large extent size charge enzyme. Both forms were...