A5048136231- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- Nerve injury and regeneration
- Neurogenesis and neuroplasticity mechanisms
- Spinal Cord Injury Research
- Neuroinflammation and Neurodegeneration Mechanisms
- Neurotransmitter Receptor Influence on Behavior
- RNA Research and Splicing
- Genetics and Neurodevelopmental Disorders
- Muscle Physiology and Disorders
- RNA modifications and cancer
- Neuroscience and Neuropharmacology Research
- Sirtuins and Resveratrol in Medicine
- Hippo pathway signaling and YAP/TAZ
- Cholinesterase and Neurodegenerative Diseases
- Histone Deacetylase Inhibitors Research
- Receptor Mechanisms and Signaling
- Cancer-related Molecular Pathways
- Tryptophan and brain disorders
- Virus-based gene therapy research
- Renin-Angiotensin System Studies
- Neurological diseases and metabolism
- Phosphodiesterase function and regulation
- Nuclear Receptors and Signaling
- Parkinson's Disease Mechanisms and Treatments
Research Institute of Health Sciences
2009-2024
Universitat de les Illes Balears
2010-2024
Health Research Institute of the Balearic Islands
2016-2021
Servei de Salut de les Illes Balears
1996-2014
Johns Hopkins University
2002-2012
Universitat de Lleida
1997-2007
Biomedical Research Institute of Lleida
2007
Johns Hopkins Medicine
2002-2003
Amyotrophic lateral sclerosis (ALS) is a progressive, lethal neuromuscular disease that associated with the degeneration of spinal and brainstem motor neurons, leading to atrophy limb, axial, respiratory muscles. The cause ALS unknown, there no effective therapy. Neurotrophic factors are candidates for therapeutic evaluation in ALS. Although chronic delivery molecules central nervous system has proven difficult, we recently discovered adeno-associated virus can be retrogradely transported...
We have investigated the potential of human pluripotent cells to restore function in rats paralyzed with a virus-induced motor neuronopathy. Cells derived from embryonic germ cells, termed embryoid body-derived (EBD) introduced into CSF were distributed extensively over rostrocaudal length spinal cord and migrated parenchyma paralyzed, but not uninjured, animals. Some transplanted expressed neuroglial progenitor marker nestin, whereas others immunohistochemical markers characteristic...
Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the selective death of motoneurons. Recently, vascular endothelial growth factor (VEGF) has been identified as neurotrophic and implicated in mechanisms pathogenesis ALS other neurological diseases. The potential neuroprotective effects VEGF rat spinal cord organotypic culture were studied model chronic glutamate excitotoxicity which transporters are inhibited threohydroxyaspartate (THA)....
Motoneuron (MN) cell death is the histopathologic hallmark of spinal muscular atrophy (SMA), although MN loss seems to be a late event. Conversely, disruption afferent synapses on MNs has been shown occur early in SMA. Using mouse model severe SMA (SMNΔ7), we examined mechanisms involved impairment central synapses. We found that underwent progressive degeneration course SMA, with still occurring at stages. Loss inputs was detected embryonic stages, long before death. Reactive microgliosis...
Disrupted in Schizophrenia-1 (DISC1) has been associated with a broad spectrum of mental disorders. DISC1 is multi-compartmentalized protein found the cytoplasm, centrosome, nuclei and mostly enriched mitochondria. In order to shed light on mitochondrial function, we have studied its topology within organelle. We show here that mammals resides ‘Mitochondrial contact site Cristae Organizing system’ (MICOS) complex, involved cristae organization. knockdown SH-SY5Y cells causes MICOS...
Vascular endothelial growth factor (VEGF), originally described as a with regulatory role in vascular and development, it is also known for its direct effects on neuronal cells. The discovery the past decade that transgenic mice expressing reduced levels of VEGF developed late-onset motoneuron pathology, reminiscent amyotrophic lateral sclerosis (ALS), opened new field research this disease. has been shown to protect motoneurons from excitotoxic death, which relevant mechanism involved...
Abstract Through GWAS studies we identified PATJ associated with functional outcome after ischemic stroke (IS). The aim of this study was to determine role in brain endothelial cells (ECs) the context outcome. expression analyses patient’s blood revealed that: (i) risk allele rs76221407 induces higher , (ii) is downregulated 24 h IS, and (iii) its significantly lower those patients independence, measured at 3 months modified Rankin scale ((mRS) ≤2), compared marked disability (mRS = 4–5). In...
Abstract We have examined the expression of glutamate transporters in primary and immortalized glial precursors (GRIPs). subsequently transduced these cells with GLT1 transporter ability to protect motor neurons an organotypic spinal cord culture. show that restricted GRIP‐derived astrocytes predominantly express GLAST GLT1. Oligodendrocyte differentiation GRIPs results downregulation all subtypes. Having identified precursor as potential vectors for delivering regions interest, we...
Acute administration of a single dose NMDA on embryonic day (E) 7 or later induces marked excitotoxic injury in the chick spinal cord, including massive necrotic motoneuron (MN) death. When same treatment was performed before E7, little, if any, response observed. Chronic with starting E5 prevents produced by "acute" NMDA. Additionally, chronic also induced non-NMDA glutamate receptor agonists, such as kainate AMPA. reduces normal MN death when is maintained during period naturally occurring...