A5034986721- Parkinson's Disease Mechanisms and Treatments
- Alzheimer's disease research and treatments
- Prion Diseases and Protein Misfolding
- Neuroscience and Neuropharmacology Research
- Neurological disorders and treatments
- Cellular transport and secretion
- Adipose Tissue and Metabolism
- Mitochondrial Function and Pathology
- Genetic Neurodegenerative Diseases
- Peroxisome Proliferator-Activated Receptors
- Genetics, Aging, and Longevity in Model Organisms
- Nerve injury and regeneration
- Botulinum Toxin and Related Neurological Disorders
- Nuclear Receptors and Signaling
- Autism Spectrum Disorder Research
- Neuroinflammation and Neurodegeneration Mechanisms
- Retinal Development and Disorders
- Genetics and Neurodevelopmental Disorders
- Neurological diseases and metabolism
- Nanoparticles: synthesis and applications
- Amyotrophic Lateral Sclerosis Research
- Advanced Glycation End Products research
- Lysosomal Storage Disorders Research
- Endoplasmic Reticulum Stress and Disease
- Aluminum toxicity and tolerance in plants and animals
Universitat Autònoma de Barcelona
2003-2024
Universitat de Vic - Universitat Central de Catalunya
2018-2024
Institut d'Investigació Biomédica de Bellvitge
2004-2016
Bellvitge University Hospital
2004-2013
Centro de Investigación Biomédica en Red
2008-2013
Centre for Biomedical Network Research on Rare Diseases
2011-2013
Universitat de Barcelona
2003-2010
Universitat de Lleida
2005-2008
Universitat de Miguel Hernández d'Elx
2006
Institut Català d'Oncologia
2006
The occurrence of endoplasmic reticulum (ER) stress in the sporadic form amyotrophic lateral sclerosis (ALS) is unknown, despite it has been recently documented experimental models familial form. Here we show that spinal cord from patients with ALS showed signs ER stress, such as increased levels chaperones protein-disulfide isomerase, and phosphorylation eukaryotic initiation factor 2α (eIF2α). Among potential causes proteasomal impairment was confirmed same samples by demonstrating...
Parkinson's disease (PD) is characterized by a progressive loss of dopaminergic neurons, process that current therapeutic approaches cannot prevent. In PD, the typical pathological hallmark accumulation intracellular protein inclusions, known as Lewy bodies and neurites, which are mainly composed α-synuclein. Here, we exploited high-throughput screening methodology to identify small molecule (SynuClean-D) able inhibit α-synuclein aggregation. SynuClean-D significantly reduces in vitro...
Diverse oxidative pathways, such as direct oxidation of amino acids, glycoxidation, and lipoxidation could contribute to Alzheimer disease pathogenesis. A global survey for the amount structurally characterized probes these reactions is lacking overcome lack specificity derived from measurement 2,4-dinitrophenylhydrazine reactive carbonyls. Consequently we analyzed (i) presence concentrations glutamic aminoadipic semialdehydes, N(epsilon)-(carboxymethyl)-lysine,...
Oxidative stress has been well documented in the substantia nigra Parkinson disease (PD), but little is known about oxidative damage, particularly lipoxidation, advanced glycation (AGE), and AGE receptors (RAGE) other structures, including cerebral cortex, early stages of diseases with Lewy bodies. The present study was undertaken to analyze these parameters frontal cortex (area 8), amygdala, selected cases no neurologic symptoms neuropathologically verified incidental body disease-related...
Tauopathies are degenerative diseases characterized by the accumulation of phosphorylated tau in neurons and glial cells. With some exceptions, deposits mainly manifested as pretangles tangles unrelated to tauopathy. It is thought that abnormal deposition occurs following specific steps, but little known about progression pathology cells tauopathies. We compared different astrocyte phenotypes oligodendroglial inclusions with a large series tauopathies, including progressive supranuclear...
Classical Rett syndrome (RTT) is a neurodevelopmental disorder where most of cases carry MECP2 mutations. Atypical RTT variants involve mutations in CDKL5 and FOXG1. However, subset patients remains that do not any mutation the described genes. Whole exome sequencing was carried out cohort 21 female probands with clinical features overlapping those RTT, but without customarily studied Candidates were functionally validated by assessing appearance neurological phenotype Caenorhabditis elegans...
Transient focal ischaemia by middle cerebral artery occlusion (MCAO) may produce cell death, but the mechanisms leading to death differ in infarct core and penumbra, immediate zone surrounding core. In present study, transient adult rats was produced intraluminal of for 1 h followed 0 ( n = 6), 10), 4 8), 6 2) 12 3) reperfusion. The model causes a large cortico‐striatal extending through mediolateral cortex dorsolateral striatum at h. expression subcellular distribution several proteins...
Mutation A30P in the alpha-synuclein gene is a cause of familial Parkinson disease. Transgenic mice expressing wild mouse and mutant human alpha-synuclein, Tg5093 (Tg), show progressive motor disorder characterized by tremor, rigidity, dystonia, accompanied accumulation soma neurites conspicuous gliosis beginning hippocampal formation at age 7 to 8 months spreading throughout CNS. Impaired short-term changes synaptic strength have also been documented slices from Tg mice. Alpha-synuclein...
Bacterial nanocellulose (BNC) is a promising dietary fiber with potential as functional food additive. We evaluated BNC fibers (BNCf) in the Caenorhabditis elegans model to obtain insight into BNCf's biointeraction its gastrointestinal tract while reducing variables of higher complex animals. BNCf were uptaken and excreted by worms without crossing intestinal barrier, confirming biosafety regarding survival rate, reproduction, aging for concentrations up 34 μg/ml BNCf. However, slight...
Hyperphosphorylated tau in neurites surrounding β‐amyloid (βA) deposits, as revealed with phospho‐specific anti‐ antibodies, are found amyloid precursor protein (APP) Tg2576 mice. Because βA is a source of oxidative stress and may be toxic for cultured cells, the present study examines expression phosphorylated (active) stress‐activated kinase c‐Jun N‐terminal (SAPK/JNK‐P) p38 (p38‐P), which have capacity to phosphorylate at specific sites, their substrates ATF‐2, involved cell death...
Parkinson's disease is associated with intracellular α-synuclein accumulation and ventral midbrain dopaminergic neuronal death in the Substantia Nigra of brain patients. The Rho GTPase pathway, mainly linking surface receptors to organization actin microtubule cytoskeletons, has been suggested participate pathogenesis. Nevertheless, its exact contribution remains obscure. To unveil participation family molecular pathogenesis disease, we first used C elegans demonstrate role small RAC1...
Diffuse Lewy body disease (DLBD) is a degenerative of the nervous system, involving brain stem, diencephalic nuclei and cerebral cortex, associated with abnormal α‐synuclein aggregation widespread formation bodies neurites. DLBD presents as pure forms (DLBDp) or in association Alzheimer (AD) common (DLBDc). Several neurotransmitter abnormalities have been reported including those nigrostriatal mesocorticolimbic dopaminergic central noradrenergic, serotoninergic cholinergic pathways. The...
α-Synuclein (α-Syn) forms toxic intracellular protein inclusions and transmissible amyloid structures in Parkinson's disease (PD). Preventing α-Syn self-assembly has become one of the most promising approaches search for disease-modifying treatments this neurodegenerative disorder. Here, we describe capacity a small molecule (ZPD-2), identified after high-throughput screening, to inhibit aggregation. ZPD-2 inhibits aggregation wild-type A30P H50Q familial variants vitro at substoichiometric...