A5074496095- Mitochondrial Function and Pathology
- Coenzyme Q10 studies and effects
- Advanced battery technologies research
- Circadian rhythm and melatonin
- Pancreatic function and diabetes
- ATP Synthase and ATPases Research
- Diet, Metabolism, and Disease
- Cannabis and Cannabinoid Research
- Sulfur Compounds in Biology
- Biochemical effects in animals
- Neuroinflammation and Neurodegeneration Mechanisms
- Autophagy in Disease and Therapy
- Calcium signaling and nucleotide metabolism
- Heart Rate Variability and Autonomic Control
- Inflammasome and immune disorders
- Folate and B Vitamins Research
- Erythrocyte Function and Pathophysiology
- Virus-based gene therapy research
- Nitric Oxide and Endothelin Effects
- Immune cells in cancer
- Oral health in cancer treatment
- Cardiovascular Function and Risk Factors
- Metabolomics and Mass Spectrometry Studies
- interferon and immune responses
- Free Radicals and Antioxidants
Universitat Autònoma de Barcelona
2021-2024
MRC Mitochondrial Biology Unit
2017-2020
University of Cambridge
2020
Universidad de Granada
2012-2018
Parque Tecnológico de la Salud
2012-2015
Mucositis is a common and distressing side effect of chemotherapy or radiotherapy that has potentially severe consequences, no treatment available. The purpose this study was to analyze the molecular pathways involved in development oral mucositis evaluate whether melatonin can prevent pathology. tongue male Wistar rats subjected irradiation (X-ray YXLON Y.Tu 320-D03 irradiator; animals received dose 7.5 Gy/day for 5 days). Rats were treated with 45 mg/day vehicle 21 days postirradiation,...
Significance Coenzyme Q (CoQ) is a requisite component of the mitochondrial oxidative phosphorylation machinery that produces more than 90% cellular ATP. Despite discovery CoQ 50 years ago, many aspects its biosynthesis remain obscure. These include functions uncharacterized CoQ-related proteins whose disruption can cause human diseases. Our work reveals one such protein, COQ9, lipid-binding protein enables through physical and functional interaction with COQ7, via stabilization entire...
Abstract Mitochondria are key cellular signaling platforms, affecting fundamental processes such as cell proliferation, differentiation and death. However, it remains unclear how mitochondrial affects other organelles, particularly lysosomes. Here, we demonstrate that respiratory chain (RC) impairments elicit a stress pathway regulates lysosomal biogenesis via the microphtalmia transcription factor family. Interestingly, effect of over depends on timeframe elicited: while RC inhibition with...
Coenzyme Q10 (CoQ10) or ubiquinone is a well-known component of the mitochondrial respiratory chain. In humans, CoQ10 deficiency causes syndrome with an unexplained variability in clinical presentations. To try to understand this heterogeneity phenotypes, we have generated Coq9 Knockin (R239X) mouse model. The lack functional protein homozygous mutant (Coq9X/X) mice severe reduction Coq7 and, as consequence, widespread CoQ and accumulation demethoxyubiquinone. deficit induces brain-specific...
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several other functions in cellular metabolism. One them to function as an electron carrier reaction catalyzed by sulfide:quinone oxidoreductase (SQR), which catalyzes first hydrogen sulfide oxidation pathway. Therefore, SQR may be affected CoQ deficiency. Using human skin fibroblasts and two mouse models with primary deficiency, we demonstrate that severe deficiency causes reduction levels activity,...
Abstract While it is accepted that the high production of nitric oxide ( NO ˙) by inducible synthase iNOS ) impairs cardiac mitochondrial function during sepsis, role neuronal nNOS may be protective. During there a significantly increase in expression and activity (i‐mt NOS ), which parallels changes cytosolic . The existence constitutive form (c‐mt heart mitochondria has been also described, but its failure sepsis remains unclear. Herein, we analyzed oxidative stress bioenergetics wild‐type...
Abstract Primary coenzyme Q 10 (CoQ ) deficiency is due to mutations in genes involved CoQ biosynthesis. The disease has been associated with five major phenotypes, but a genotype–phenotype correlation unclear. Here, we compare two mouse models genetic modification Coq9 gene ( Q95X and R239X ), their responses 2,4‐dihydroxybenzoic acid (2,4‐di HB ). mice manifest severe widespread fatal encephalomyopathy respond 2,4‐di increasing levels. In contrast, exhibit mild manifesting reduction CI +...
Leigh syndrome, or infantile necrotizing subacute encephalopathy (OMIM #256000), is one of the most common manifestations mitochondrial dysfunction, due to mutations in more than 75 genes, with respiratory complex I subunits being cause. In present study, we used recently described PHP.B serotype, characterized by efficient capacity cross blood-brain barrier, express
Primary coenzyme Q10 (CoQ10) deficiency is a rare mitochondrial disorder associated with 5 major clinical phenotypes: (1) encephalomyopathy, (2) severe infantile multisystemic disease, (3) cerebellar ataxia, (4) isolated myopathy, and (5) steroid-resistant nephrotic syndrome. Growth retardation, deafness hearing loss have also been described in CoQ10-deficient patients. This heterogeneity the presentations suggests that multiple pathomechanisms may exist. To investigate biochemical molecular...
Abstract Mitochondrial dysfunction and oxidative/nitrosative stress are common features of senescence, they explain some the pathophysiological events during aging. In different animal models aging, existence oxidative stress, inflammation, mitochondrial has been reported. There is no information, however, regarding age when these symptoms begin if account for gender differences in Here we analyzed markers bioenergetics brain mitochondria normal mice first 10 months life, looking early signs...
The powerful antioxidant capacity of virgin argan oil is attributed to its content molecules. Recent investigations have identified CoQ10 and melatonin as some these In this review, we summarize the most recent data about in differences found samples extracted by traditional half-industrialized methods. We also emphasize importance two molecules for human health, focusing on their actions mitochondria. Finally, refer other abundant antioxidants oil: tocopherols polyphenols.
Moderate overexpression of Opa1, the master regulator mitochondrial cristae morphology, significantly improved damage induced by drugs, surgical denervation, or oxidative phosphorylation (OXPHOS) defects due to specific impairment a single respiratory chain complex. Here, we investigated effectiveness this approach in Mpv17−/− mouse, characterized profound, multisystem DNA (mtDNA) depletion. After crossing with Opa1tg mice, found surprising anticipation severe, progressive focal segmental...
Mitohormesis is an adaptive response induced by a mild mitochondrial stress that promotes longevity and metabolic health in different organisms. This mechanism has been proposed as the cause of increase survival Coq7+/- (Mclk1+/-) mice, which show hepatic reduction COQ7, early dysfunction increased oxidative stress. Our study shows lack COQ9 Coq9Q95X mice triggers COQ6 COQ5, results life expectancy. However, our reveal CoQ levels are not decreased and, therefore, neither or observed liver...
Abstract Moderate overexpression of Opa1 , encoding a master regulator mitochondrial cristae morphology, has been shown to improve significantly damage induced by drugs, surgical denervation, or genetically determined OXPHOS defects. However, this approach so far demonstrated in limited number defective models characterized specific impairment single respiratory chain complex. Here, we investigated the effectiveness moderate Mpv17 -/- mouse, profound, multisystem mtDNA depletion. In naïve...
<title>Abstract</title> Mutations in mitochondrial energy-producing genes lead to a heterogeneous group of untreatable disorders known as primary diseases (MD). Leigh syndrome (LS) is the most common pediatric MD and characterized by progressive neuromuscular affectation premature death. Here, we show that daily cannabidiol (CBD) administration significantly extends lifespan ameliorates pathology two LS mouse models, cellular function fibroblast from patients. CBD delays motor decline...