A5031611015- Mitochondrial Function and Pathology
- Adipose Tissue and Metabolism
- Genetics, Aging, and Longevity in Model Organisms
- Metabolomics and Mass Spectrometry Studies
- Peroxisome Proliferator-Activated Receptors
- Diet and metabolism studies
- Alzheimer's disease research and treatments
- Antioxidant Activity and Oxidative Stress
- Biochemical effects in animals
- Fatty Acid Research and Health
- Neuroinflammation and Neurodegeneration Mechanisms
- Coenzyme Q10 studies and effects
- Physiological and biochemical adaptations
- Folate and B Vitamins Research
- Endoplasmic Reticulum Stress and Disease
- Metabolism and Genetic Disorders
- Advanced Glycation End Products research
- Tryptophan and brain disorders
- Parkinson's Disease Mechanisms and Treatments
- RNA regulation and disease
- Biochemical Acid Research Studies
- Alcoholism and Thiamine Deficiency
- Lipid metabolism and disorders
- Cardiac Ischemia and Reperfusion
- Amyotrophic Lateral Sclerosis Research
Universitat de Lleida
2014-2024
Biomedical Research Institute of Lleida
2013-2022
Institute for Research in Biomedicine
2016-2019
University of Cambridge
2018
Universitat de Barcelona
2008-2011
Institut d'Investigació Biomédica de Bellvitge
2008-2011
Bellvitge University Hospital
2008-2011
Centro de Investigación Biomédica en Red
2011
Biomedical Research Networking Center on Neurodegenerative Diseases
2008
X-linked adrenoleukodystrophy (X-ALD) is a fatal neurodegenerative disorder, characterized by progressive cerebral demyelination childhood (CCALD) or spinal cord neurodegeneration (adrenomyeloneuropathy, AMN), adrenal insufficiency and accumulation of very long-chain fatty acids (VLCFA) in tissues. The disease caused mutations the ABCD1 gene, which encodes peroxisomal transporter that plays role import VLCFA VLCFA–CoA into peroxisomes. Abcd1 knockout mice develop mimics AMN adult patients,...
Mutations in mitochondrial oxidative phosphorylation complex I are associated with multiple pathologies, and has been proposed as a crucial regulator of animal longevity. In yeast, the single-subunit NADH dehydrogenase Ndi1 serves non-proton-translocating alternative enzyme that replaces I, bringing about reoxidation intramitochondrial NADH. We have created transgenic strains Drosophila express yeast NDI1 ubiquitously. Mitochondrial extracts from NDI1-expressing flies displayed...
Axonal degeneration is a main contributor to disability in progressive neurodegenerative diseases which oxidative stress often identified as pathogenic factor. We aim demonstrate that antioxidants are able improve axonal and locomotor deficits mouse model of X-adrenoleukodystrophy (X-ALD).X-ALD lethal disease caused by loss function the ABCD1 peroxisomal transporter very long chain fatty acids (VLCFA). The for X-ALD exhibits late onset neurological phenotype with spinal cord resembling most...
Caloric restriction (CR) decreases oxidative damage, which contributes to the slowing of aging rate. It is not known if such are due calories themselves or specific dietary components. In this work, ingestion proteins Wistar rats was decreased by 40% below that controls. After 7 weeks, liver protein-restricted (PR) animals showed in protein degree membrane unsaturation, and mitochondrial complex I content. The results previous information suggest decrease rate induced PR can be part reactive...
Eighty percent dietary methionine restriction (MetR) in rodents (without calorie restriction), like (DR), increases maximum longevity and strongly decreases mitochondrial reactive oxygen species (ROS) production oxidative stress. MetR also lowers the degree of membrane fatty acid unsaturation rat liver. Mitochondrial ROS generation are only two known factors linking stress with vertebrates. However, it is unknown whether 40% MetR, relevant to clarify mechanisms action standard (40%) DR can...
X-linked adrenoleukodystrophy (X-ALD) is a fatal, axonal demyelinating, neurometabolic disease. It results from the functional loss of member peroxisomal ATP-binding cassette transporter subfamily D (ABCD1), which involved in metabolism very long-chain fatty acids (VLCFA). Oxidative damage proteins caused by excess hexacosanoic acid, most prevalent VLCFA accumulating X-ALD, an early event neurodegenerative cascade. We demonstrate here that valproic acid (VPA), widely used anti-epileptic drug...
A molecular description of the mechanisms by which aging is produced still very limited. Here, we have determined plasma metabolite profile using high-throughput metabolome profiling technologies 150 healthy humans ranging from 30 to 100 years age. Using a nontargeted approach, detected 2,678 species in plasma, and multivariate analyses separated perfectly two groups indicating specific signature for each gender. In addition, there set gender-shared metabolites, change significantly during...
Chronic metabolic impairment and oxidative stress are associated with the pathogenesis of axonal dysfunction in a growing number neurodegenerative conditions. To investigate intertwining both noxious factors, we have chosen mouse model adrenoleukodystrophy (X-ALD), which exhibits degeneration spinal cords motor disability. The disease is caused by loss function ABCD1 transporter, involved import degradation very long-chain fatty acids (VLCFA) peroxisomes. Oxidative due to VLCFA excess...
Lipid composition, particularly membrane unsaturation, has been proposed as being a lifespan determinant, but it is currently unknown whether caloric restriction (CR), an accepted life-extending intervention, affects cellular lipid profiles. In this study, we employ liquid chromatography quadrupole time-of-flight-based methodology to demonstrate that CR in the liver of male C57BL/6 mice: (i) induces marked changes lipidome, (ii) specifically reduces levels phospholipid peroxidation product,...
X-linked adrenoleukodystrophy is a neurometabolic disorder caused by inactivation of the peroxisomal ABCD1 transporter very long-chain fatty acids. In mice, loss causes late onset axonal degeneration in spinal cord association with locomotor disability resembling most common phenotype patients, adrenomyeloneuropathy. Increasing evidence indicates that oxidative stress and bioenergetic failure play major roles pathogenesis adrenoleukodystrophy. this study, we aimed to evaluate whether...
Tau P301S transgenic mice (PS19 line) are used as a model of frontotemporal lobar degeneration (FTLD)-tau. Behavioral alterations in these begin at approximately 4 months age. We analyzed molecular changes related to disease progression mice. Hyperphosphorylated 4Rtau increased neurons from 1 month age entorhinal and piriform cortices the neocortex other regions. A small percentage developed an abnormal tau conformation, truncation, ubiquitination only 9/10 Astrocytosis, microgliosis,...
Plasma lipidomic profile is species specific and an optimized feature associated with animal longevity. In the present work, use of mass spectrometry technologies allowed us to determine plasma fatty acid pattern healthy humans exceptional Here, we show that it possible define a signature only using 20 lipid discriminate adult, aged centenarian subjects obtaining almost perfect accuracy (90%-100%). Furthermore, propose belonging ceramides, widely involved in cell-stress response, as...
Aging plays a central role in the occurrence of neurodegenerative diseases. Caloric restriction (CR) mitigates oxidative stress by decreasing rate generation endogenous damage, mechanism that can contribute to slowing aging induced this intervention. Various reports have recently linked methionine aging, and (MetR) without energy also increases life span. We thus hypothesized MetR be responsible, at least part, for decrease damage CR. In investigation we subjected male rats exactly same...