Daniel Jose
Moreno Fernandez-Ayala
Publications (42) Daniel Jose Moreno Fernandez-Ayala publications
2024
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Mutations of GEMIN5 are associated with coenzyme Q10 deficiency: long-term follow-up after treatment
European Journal of Human Genetics, Vol. 32, Núm. 4, pp. 426-434
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New variants expand the neurological phenotype of COQ7 deficiency
Journal of Inherited Metabolic Disease, Vol. 47, Núm. 5, pp. 1047-1068
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Prenatal and progressive coenzyme Q10 administration to mitigate muscle dysfunction in mitochondrial disease
Journal of Cachexia, Sarcopenia and Muscle
2022
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Calorie Restriction Rescues Mitochondrial Dysfunction in Adck2-Deficient Skeletal Muscle
Frontiers in Physiology, Vol. 13
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Isolation of Mitochondria from Mouse Skeletal Muscle for Respirometric Assays
Journal of Visualized Experiments, Vol. 2022, Núm. 180
2021
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Coenzyme q at the hinge of health and metabolic diseases
Antioxidants, Vol. 10, Núm. 11
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Model cells and organisms in mitochondrial diseases
Mitochondrial Diseases: Theory, Diagnosis and Therapy (Springer International Publishing), pp. 231-271
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Secondary CoQ10 deficiency, bioenergetics unbalance in disease and aging
BioFactors, Vol. 47, Núm. 4, pp. 551-569
2020
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Age-related mitochondrial dysfunction as a key factor in COVID-19 disease
Experimental Gerontology, Vol. 142
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Coenzyme Q and Aging in the Fruit Fly Drosophila melanogaster
Coenzyme Q in Aging (Springer International Publishing), pp. 141-155
2019
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ADCK2 haploinsufficiency reduces mitochondrial lipid oxidation and causes myopathy associated with CoQ deficiency
Journal of Clinical Medicine, Vol. 8, Núm. 9
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Essential Physiological Differences Characterize Short- and Long-Lived Strains of Drosophila melanogaster
Journals of Gerontology - Series A Biological Sciences and Medical Sciences, Vol. 74, Núm. 12, pp. 1835-1843
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Study of the genotype-phenotype correlation in fibroblasts of patients with mutations in COQ4
Biosaia: Revista de los másteres de Biotecnología Sanitaria y Biotecnología Ambiental, Industrial y Alimentaria, Núm. 8
2018
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Mitochondrial dysfunction in metabolism and ageing: shared mechanisms and outcomes?
Biogerontology, Vol. 19, Núm. 6, pp. 461-480
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The mitochondrial phosphatase PPTC7 orchestrates mitochondrial metabolism regulating coenzyme Q10 biosynthesis
Biochimica et Biophysica Acta - Bioenergetics, Vol. 1859, Núm. 11, pp. 1235-1248
2017
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Biochemical assessment of coenzyme Q10 deficiency
Journal of Clinical Medicine, Vol. 6, Núm. 3
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Genetic Rescue of Mitochondrial and Skeletal Muscle Impairment in an Induced Pluripotent Stem Cells Model of Coenzyme Q10 Deficiency
Stem Cells, Vol. 35, Núm. 7, pp. 1687-1703
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Role of mitochondrial reverse electron transport in ROS signaling: Potential roles in health and disease
Frontiers in Physiology, Vol. 8, Núm. JUN
2016
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Mitochondrial ROS Produced via Reverse Electron Transport Extend Animal Lifespan
Cell Metabolism, Vol. 23, Núm. 4, pp. 725-734
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RNA-binding proteins regulate cell respiration and coenzyme Q biosynthesis by post-transcriptional regulation of COQ7
RNA Biology, Vol. 13, Núm. 7, pp. 622-634