List of works - Luis C. López

A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial disease

scientific article

A review of the melatonin functions in zebrafish physiology

scientific article

ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency

scientific article

Acute and chronic mitochondrial respiratory chain deficiency differentially regulate lysosomal biogenesis.

scientific article published on 27 March 2017

Age-dependent lipopolysaccharide-induced iNOS expression and multiorgan failure in rats: Effects of melatonin treatment

scientific article published on 17 October 2006

Analysis of the daily changes of melatonin receptors in the rat liver

scientific article published on 30 October 2012

Antioxidant effect of exercise: Exploring the role of the mitochondrial complex I superassembly.

scientific article published on 11 July 2017

Argan oil-contained antioxidants for human mitochondria.

scientific article published on January 2013

Assessment of thymidine phosphorylase function: measurement of plasma thymidine (and deoxyuridine) and thymidine phosphorylase activity

scientific article published on January 2012

Attenuation of cardiac mitochondrial dysfunction by melatonin in septic mice

scientific article published on 20 March 2007

Bypassing human CoQ10 deficiency

scientific article published on 9 December 2017

Cellular mechanisms involved in the melatonin inhibition of HT-29 human colon cancer cell proliferation in culture

scientific article published on 01 September 2007

Chronic melatonin treatment prevents age-dependent cardiac mitochondrial dysfunction in senescence-accelerated mice.

scientific article published in January 2007

Chronic melatonin treatment reduces the age-dependent inflammatory process in senescence-accelerated mice

scientific article published in April 2007

Clinical and genetic analysis of lipid storage myopathies

scientific article published in March 2009

CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome.

scientific article

CoQ10 supplementation rescues nephrotic syndrome through normalization of H2S oxidation pathway

scientific article published on 06 September 2018

Combination of melatonin and rapamycin for head and neck cancer therapy: Suppression of AKT/mTOR pathway activation, and activation of mitophagy and apoptosis via mitochondrial function regulation

scientific article published on 16 December 2017

Composition and Antioxidant Properties of Spanish Extra Virgin Olive Oil Regarding Cultivar, Harvest Year and Crop Stage

scientific article published on 11 July 2019

Cord blood-derived CD34+ hematopoietic cells with low mitochondrial mass are enriched in hematopoietic repopulating stem cell function.

scientific article published on 24 January 2013

Deoxypyrimidine monophosphate bypass therapy for thymidine kinase 2 deficiency.

scientific article

Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics.

scientific article published on 20 May 2017

Determination of Coenzyme Q10, Coenzyme Q9, and Melatonin Contents in Virgin Argan Oils: Comparison with Other Edible Vegetable Oils

scientific article published on 31 October 2011

Disruption of the NF-κB/NLRP3 connection by melatonin requires retinoid-related orphan receptor-α and blocks the septic response in mice

scientific article published on 4 June 2015

Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency

scientific journal article

ETFDH mutations, CoQ10 levels, and respiratory chain activities in patients with riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency

scientific article (publication date: March 2009)

Early gender differences in the redox status of the brain mitochondria with age: effects of melatonin therapy.

scientific article published in December 2013

Extrapineal melatonin: analysis of its subcellular distribution and daily fluctuations

scientific article published on 2 September 2011

Extrapineal melatonin: sources, regulation, and potential functions.

scientific article published on 20 February 2014

Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9R239X Mice.

scientific article

Heterogeneity of coenzyme Q10 deficiency: patient study and literature review

scientific article published on August 2012

Hydroxytyrosol influences exercise-induced mitochondrial respiratory complex assembly into supercomplexes in rats

scientific article published on 24 January 2019

Identification of an inducible nitric oxide synthase in diaphragm mitochondria from septic mice: its relation with mitochondrial dysfunction and prevention by melatonin.

scientific article

Identification of mitochondrial deficits and melatonin targets in liver of septic mice by high-resolution respirometry.

scientific article published on 10 December 2014

Identification of morphological markers of sarcopenia at early stage of aging in skeletal muscle of mice.

scientific article published on 16 July 2016

Improved mitochondrial function and increased life span after chronic melatonin treatment in senescent prone mice.

scientific article published on 6 April 2008

In Vivo Determination of Mitochondrial Respiration in 1-Methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine-Treated Zebrafish Reveals the Efficacy of Melatonin in Restoring Mitochondrial Normalcy.

scientific article published on 29 November 2017

Inhibition of neuronal nitric oxide synthase activity by N1-acetyl-5-methoxykynuramine, a brain metabolite of melatonin

scientific article published in September 2006

Lack of NLRP3 Inflammasome Activation Reduces Age-Dependent Sarcopenia and Mitochondrial Dysfunction, Favoring the Prophylactic Effect of Melatonin

scientific article published on 01 October 2019

Lack of aprataxin impairs mitochondrial functions via downregulation of the APE1/NRF1/NRF2 pathway.

scientific article published on 14 May 2015

Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations

scientific article

Long-term melatonin administration protects brain mitochondria from aging.

scientific article published in July 2009

Mechanisms of N-methyl-D-aspartate receptor inhibition by melatonin in the rat striatum

scientific article published in November 2004

Melatonin Enhances Cisplatin and Radiation Cytotoxicity in Head and Neck Squamous Cell Carcinoma by Stimulating Mitochondrial ROS Generation, Apoptosis, and Autophagy

scientific article published on 28 February 2019

Melatonin administration to wild-type mice and nontreated NLRP3 mutant mice share similar inhibition of the inflammatory response during sepsis.

scientific article published on 31 March 2017

Melatonin and its brain metabolite N(1)-acetyl-5-methoxykynuramine prevent mitochondrial nitric oxide synthase induction in parkinsonian mice.

scientific article published on October 2009

Melatonin blunts the mitochondrial/NLRP3 connection and protects against radiation-induced oral mucositis

scientific article

Melatonin counteracts inducible mitochondrial nitric oxide synthase-dependent mitochondrial dysfunction in skeletal muscle of septic mice.

scientific article published in January 2006

Melatonin enhances neural stem cell differentiation and engraftment by increasing mitochondrial function.

scientific article

Melatonin plus physical exercise are highly neuroprotective in the 3xTg-AD mouse

scientific article

Melatonin protects lung mitochondria from aging ⬇️

scientific article published on May 26, 2011

Melatonin protects rats from radiotherapy-induced small intestine toxicity

scientific article

Melatonin rescues zebrafish embryos from the parkinsonian phenotype restoring the parkin/PINK1/DJ-1/MUL1 network

scientific article published on 11 April 2016

Melatonin restores the mitochondrial production of ATP in septic mice

scientific article published on 01 October 2006

Melatonin role in the mitochondrial function.

scientific article published on January 2007

Melatonin treatment counteracts the hyperoxidative status in erythrocytes of patients suffering from Duchenne muscular dystrophy

scientific article published on 15 April 2011

Melatonin treatment normalizes plasma pro-inflammatory cytokines and nitrosative/oxidative stress in patients suffering from Duchenne muscular dystrophy

scientific article published on 4 March 2010

Melatonin, clock genes and mitochondria in sepsis.

scientific article published on 07 August 2017

Mitochondrial DNA and inflammatory diseases.

scientific article published on 07 July 2011

Mitochondrial impairment and melatonin protection in parkinsonian mice do not depend of inducible or neuronal nitric oxide synthases

scientific article published on 11 August 2017

Oxidative stress status, clinical outcome, and β-globin gene cluster haplotypes in pediatric patients with sickle cell disease.

scientific article published on December 2010

Pathomechanisms in coenzyme q10-deficient human fibroblasts

scientific article

Permeabilized myocardial fibers as model to detect mitochondrial dysfunction during sepsis and melatonin effects without disruption of mitochondrial network

scientific article

Protective effects of melatonin against oxidative damage induced by Egyptian cobra (Naja haje) crude venom in rats

scientific article published on 24 December 2014

Protective effects of synthetic kynurenines on 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced parkinsonism in mice

scientific article published on 17 March 2011

Rapamycin administration is not a valid therapeutic strategy for every case of mitochondrial disease

scientific article published on 18 March 2019

Reduction in the levels of CoQ biosynthetic proteins is related to an increase in lifespan without evidence of hepatic mitohormesis

scientific article published in Scientific Reports

Same molecule but different expression: aging and sepsis trigger NLRP3 inflammasome activation, a target of melatonin.

scientific article published on 17 December 2015

Synergism between melatonin and atorvastatin against endothelial cell damage induced by lipopolysaccharide

scientific article published on 17 May 2011

The Protective Effect of Melatonin Against Age-Associated Sarcopenia-Dependent Tubular Aggregates Formation, Lactate Depletion and Mitochondrial Changes.

scientific article published on 19 March 2018

The Q-junction and the inflammatory response are critical pathological and therapeutic factors in CoQ deficiency

The Role of Sulfide Oxidation Impairment in the Pathogenesis of Primary CoQ Deficiency

scientific article published on 25 July 2017

The beneficial effects of melatonin against heart mitochondrial impairment during sepsis: inhibition of iNOS and preservation of nNOS.

scientific article

The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene.

scientific article published on 23 March 2015

Thymidine and deoxyuridine accumulate in tissues of patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)

scientific article

Thymidine kinase 2 (H126N) knockin mice show the essential role of balanced deoxynucleotide pools for mitochondrial DNA maintenance.

scientific article

Treatment of CoQ10 Deficient Fibroblasts with Ubiquinone, CoQ Analogs, and Vitamin C: Time- and Compound-Dependent Effects ⬇️

scientific article published on July 30, 2010

Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency

scientific article published on 24 February 2014

β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in mice

scientific article published on 01 January 2019

List of works by Luis C. López

A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial disease

A review of the melatonin functions in zebrafish physiology

ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency

Acute and chronic mitochondrial respiratory chain deficiency differentially regulate lysosomal biogenesis.

Age-dependent lipopolysaccharide-induced iNOS expression and multiorgan failure in rats: Effects of melatonin treatment

Analysis of the daily changes of melatonin receptors in the rat liver

Antioxidant effect of exercise: Exploring the role of the mitochondrial complex I superassembly.

Argan oil-contained antioxidants for human mitochondria.

Assessment of thymidine phosphorylase function: measurement of plasma thymidine (and deoxyuridine) and thymidine phosphorylase activity

Attenuation of cardiac mitochondrial dysfunction by melatonin in septic mice

Bypassing human CoQ10 deficiency

Cellular mechanisms involved in the melatonin inhibition of HT-29 human colon cancer cell proliferation in culture

Chronic melatonin treatment prevents age-dependent cardiac mitochondrial dysfunction in senescence-accelerated mice.

Chronic melatonin treatment reduces the age-dependent inflammatory process in senescence-accelerated mice

Clinical and genetic analysis of lipid storage myopathies

CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome.

CoQ10 supplementation rescues nephrotic syndrome through normalization of H2S oxidation pathway

Combination of melatonin and rapamycin for head and neck cancer therapy: Suppression of AKT/mTOR pathway activation, and activation of mitophagy and apoptosis via mitochondrial function regulation

Composition and Antioxidant Properties of Spanish Extra Virgin Olive Oil Regarding Cultivar, Harvest Year and Crop Stage

Cord blood-derived CD34+ hematopoietic cells with low mitochondrial mass are enriched in hematopoietic repopulating stem cell function.

Deoxypyrimidine monophosphate bypass therapy for thymidine kinase 2 deficiency.

Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics.

Determination of Coenzyme Q10, Coenzyme Q9, and Melatonin Contents in Virgin Argan Oils: Comparison with Other Edible Vegetable Oils

Disruption of the NF-κB/NLRP3 connection by melatonin requires retinoid-related orphan receptor-α and blocks the septic response in mice

Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency

ETFDH mutations, CoQ10 levels, and respiratory chain activities in patients with riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency

Early gender differences in the redox status of the brain mitochondria with age: effects of melatonin therapy.

Extrapineal melatonin: analysis of its subcellular distribution and daily fluctuations

Extrapineal melatonin: sources, regulation, and potential functions.

Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9R239X Mice.

Heterogeneity of coenzyme Q10 deficiency: patient study and literature review

Hydroxytyrosol influences exercise-induced mitochondrial respiratory complex assembly into supercomplexes in rats

Identification of an inducible nitric oxide synthase in diaphragm mitochondria from septic mice: its relation with mitochondrial dysfunction and prevention by melatonin.

Identification of mitochondrial deficits and melatonin targets in liver of septic mice by high-resolution respirometry.

Identification of morphological markers of sarcopenia at early stage of aging in skeletal muscle of mice.

Improved mitochondrial function and increased life span after chronic melatonin treatment in senescent prone mice.

In Vivo Determination of Mitochondrial Respiration in 1-Methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine-Treated Zebrafish Reveals the Efficacy of Melatonin in Restoring Mitochondrial Normalcy.

Inhibition of neuronal nitric oxide synthase activity by N1-acetyl-5-methoxykynuramine, a brain metabolite of melatonin

Lack of NLRP3 Inflammasome Activation Reduces Age-Dependent Sarcopenia and Mitochondrial Dysfunction, Favoring the Prophylactic Effect of Melatonin

Lack of aprataxin impairs mitochondrial functions via downregulation of the APE1/NRF1/NRF2 pathway.

Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations

Long-term melatonin administration protects brain mitochondria from aging.

Mechanisms of N-methyl-D-aspartate receptor inhibition by melatonin in the rat striatum

Melatonin Enhances Cisplatin and Radiation Cytotoxicity in Head and Neck Squamous Cell Carcinoma by Stimulating Mitochondrial ROS Generation, Apoptosis, and Autophagy

Melatonin administration to wild-type mice and nontreated NLRP3 mutant mice share similar inhibition of the inflammatory response during sepsis.

Melatonin and its brain metabolite N(1)-acetyl-5-methoxykynuramine prevent mitochondrial nitric oxide synthase induction in parkinsonian mice.

Melatonin blunts the mitochondrial/NLRP3 connection and protects against radiation-induced oral mucositis

Melatonin counteracts inducible mitochondrial nitric oxide synthase-dependent mitochondrial dysfunction in skeletal muscle of septic mice.

Melatonin enhances neural stem cell differentiation and engraftment by increasing mitochondrial function.

Melatonin plus physical exercise are highly neuroprotective in the 3xTg-AD mouse

Melatonin protects lung mitochondria from aging ⬇️

Melatonin protects rats from radiotherapy-induced small intestine toxicity

Melatonin rescues zebrafish embryos from the parkinsonian phenotype restoring the parkin/PINK1/DJ-1/MUL1 network

Melatonin restores the mitochondrial production of ATP in septic mice

Melatonin role in the mitochondrial function.

Melatonin treatment counteracts the hyperoxidative status in erythrocytes of patients suffering from Duchenne muscular dystrophy

Melatonin treatment normalizes plasma pro-inflammatory cytokines and nitrosative/oxidative stress in patients suffering from Duchenne muscular dystrophy

Melatonin, clock genes and mitochondria in sepsis.

Mitochondrial DNA and inflammatory diseases.

Mitochondrial impairment and melatonin protection in parkinsonian mice do not depend of inducible or neuronal nitric oxide synthases

Oxidative stress status, clinical outcome, and β-globin gene cluster haplotypes in pediatric patients with sickle cell disease.

Pathomechanisms in coenzyme q10-deficient human fibroblasts

Permeabilized myocardial fibers as model to detect mitochondrial dysfunction during sepsis and melatonin effects without disruption of mitochondrial network

Protective effects of melatonin against oxidative damage induced by Egyptian cobra (Naja haje) crude venom in rats

Protective effects of synthetic kynurenines on 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced parkinsonism in mice

Rapamycin administration is not a valid therapeutic strategy for every case of mitochondrial disease

Reduction in the levels of CoQ biosynthetic proteins is related to an increase in lifespan without evidence of hepatic mitohormesis

Same molecule but different expression: aging and sepsis trigger NLRP3 inflammasome activation, a target of melatonin.

Synergism between melatonin and atorvastatin against endothelial cell damage induced by lipopolysaccharide

The Protective Effect of Melatonin Against Age-Associated Sarcopenia-Dependent Tubular Aggregates Formation, Lactate Depletion and Mitochondrial Changes.

The Q-junction and the inflammatory response are critical pathological and therapeutic factors in CoQ deficiency

The Role of Sulfide Oxidation Impairment in the Pathogenesis of Primary CoQ Deficiency

The beneficial effects of melatonin against heart mitochondrial impairment during sepsis: inhibition of iNOS and preservation of nNOS.

The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene.

Thymidine and deoxyuridine accumulate in tissues of patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)

Thymidine kinase 2 (H126N) knockin mice show the essential role of balanced deoxynucleotide pools for mitochondrial DNA maintenance.

Treatment of CoQ10 Deficient Fibroblasts with Ubiquinone, CoQ Analogs, and Vitamin C: Time- and Compound-Dependent Effects ⬇️

Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency

β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in mice