A5014289292- Coenzyme Q10 studies and effects
- Mitochondrial Function and Pathology
- Advanced battery technologies research
- Biochemical Acid Research Studies
- Adipose Tissue and Metabolism
- Vitamin C and Antioxidants Research
- Genetics, Aging, and Longevity in Model Organisms
- Sirtuins and Resveratrol in Medicine
- Metabolism and Genetic Disorders
- ATP Synthase and ATPases Research
- Plant Stress Responses and Tolerance
- Muscle metabolism and nutrition
- Biochemical effects in animals
- Diet and metabolism studies
- Aldose Reductase and Taurine
- Genomics, phytochemicals, and oxidative stress
- Photosynthetic Processes and Mechanisms
- Adenosine and Purinergic Signaling
- Lipid Membrane Structure and Behavior
- Plant nutrient uptake and metabolism
- Endoplasmic Reticulum Stress and Disease
- Autophagy in Disease and Therapy
- Exercise and Physiological Responses
- Sphingolipid Metabolism and Signaling
- Free Radicals and Antioxidants
Universidad Pablo de Olavide
2016-2025
Centro Andaluz de Biología del Desarrollo
2016-2025
Instituto de Salud Carlos III
2016-2025
Universidad de Navarra
2025
Junta de Andalucía
2008-2024
Centre for Biomedical Network Research on Rare Diseases
2015-2024
Centro de Biología Molecular Severo Ochoa
2024
Universidad Autónoma de Madrid
2015-2024
Consejo Superior de Investigaciones Científicas
2011-2023
Instituto de Investigación de Enfermedades Raras
2008-2020
The textbook description of mitochondrial respiratory complexes (RCs) views them as free-moving entities linked by the mobile carriers coenzyme Q (CoQ) and cytochrome c (cyt c). This model (known fluid model) is challenged proposal that all RCs except complex II can associate in supercomplexes (SCs). proposed SCs are respirasome (complexes I, III, IV), I III IV. role unclear, their existence debated. By genetic modulation interactions between IV, we show these associations define dedicated...
Age-related accumulation of cellular damage and death has been linked to oxidative stress. Calorie restriction (CR) is the most robust, nongenetic intervention that increases lifespan reduces rate aging in a variety species. Mechanisms responsible for antiaging effects CR remain uncertain, but reduction stress within mitochondria remains major focus research. hypothesized decrease mitochondrial electron flow proton leaks attenuate caused by reactive oxygen We have focused our research on...
Steroid-resistant nephrotic syndrome (SRNS) is a frequent cause of end-stage renal failure. Identification single-gene causes SRNS has generated some insights into its pathogenesis; however, additional genes and disease mechanisms remain obscure, continues to be treatment refractory. Here we have identified 6 different mutations in coenzyme Q10 biosynthesis monooxygenase (COQ6) 13 individuals from 7 families by homozygosity mapping. Each mutation was linked early-onset with sensorineural...
Increased production of reactive oxygen species (ROS) has long been considered a cause aging. However, recent studies have implicated ROS as essential secondary messengers. Here we show that the site significantly contributes to their apparent dual nature. We report increase with age mitochondrial function deteriorates. also demonstrate increasing specifically through respiratory complex I reverse electron transport extends Drosophila lifespan. Reverse rescued pathogenesis induced by severe...
Electrons feed into the mitochondrial electron transport chain (mETC) from NAD- or FAD-dependent enzymes. A shift glucose to fatty acids increases flux through FAD, which can saturate oxidation capacity of dedicated coenzyme Q (CoQ) pool and result in generation reactive oxygen species. To prevent this, mETC superstructure be reconfigured degradation respiratory complex I, liberating associated III increase via FAD at expense NAD. Here, we demonstrate that this adaptation is driven by ratio...
Caloric restriction (CR) is the most potent intervention known to both protect against carcinogenesis and extend lifespan in laboratory animals. A variety of anticarcinogens CR mimetics induce activate NF-E2-related factor 2 (Nrf2) pathway. Nrf2, turn, induces a number antioxidative carcinogen-detoxifying enzymes. Thus, Nrf2 offers promising target for anticarcinogenesis antiaging interventions. We used Nrf2-disrupted (KO) mice examine its role on biological effects CR. Here, we show that...
Coenzyme Q10 (CoQ10) deficiency has been associated with various clinical phenotypes, including an infantile multisystem disorder. The authors report a 33-month-old boy who presented corticosteroid-resistant nephrotic syndrome in whom progressive encephalomyopathy later developed. CoQ10 was decreased both muscle and fibroblasts. Oral improved the neurologic picture but not renal dysfunction.
Coenzyme Q10 (CoQ) is a small lipophilic molecule critical for the transport of electrons from complexes I and II to complex III in mitochondrial respiratory chain. CoQ deficiency rare human genetic condition that has been associated with variety clinical phenotypes. With aim elucidating how affects an organism, we have investigated pathophysiologic processes present within fibroblasts derived 4 patients deficiency. Assays cultured revealed decreased activities II+III, III, IV, reduced...
Coenzyme Q(10) (CoQ(10)) is essential for electron transport in the mitochondrial respiratory chain and antioxidant defense. Last year, we reported first mutations CoQ(10) biosynthetic genes, COQ2, which encodes 4-parahydroxybenzoate: polyprenyl transferase; PDSS2, subunit 2 of decaprenyl diphosphate synthase. However, pathogenic mechanisms primary deficiency have not been well characterized. In this study, investigated consequence severe on bioenergetics, oxidative stress, defenses cultured...
Abstract Introduction Fibromyalgia is a chronic pain syndrome with unknown etiology. Recent studies have shown some evidence demonstrating that oxidative stress may role in the pathophysiology of fibromyalgia. However, it still not clear whether cause or effect abnormalities documented Furthermore, mitochondria redox imbalance reported fibromyalgia also controversial. We undertook this study to investigate mitochondrial dysfunction, stress, and mitophagy Methods studied 20 patients (2 male,...
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a mitochondrial disease most usually caused by point mutations in tRNA genes encoded mtDNA. Here, we report on how this mutation affects function primary fibroblast cultures established from 2 patients with MELAS who harbored the A3243G mutation. Both respiratory chain enzyme activities coenzyme Q(10) (CoQ) levels were significantly decreased fibroblasts. A similar decrease membrane potential was found...