Publicaciones (42) Publicaciones de Daniel Jose Moreno Fernandez-Ayala

2024

  1. 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

  2. New variants expand the neurological phenotype of COQ7 deficiency

    Journal of Inherited Metabolic Disease

  3. Prenatal and progressive coenzyme Q10 administration to mitigate muscle dysfunction in mitochondrial disease

    Journal of Cachexia, Sarcopenia and Muscle

2022

  1. Calorie Restriction Rescues Mitochondrial Dysfunction in Adck2-Deficient Skeletal Muscle

    Frontiers in Physiology, Vol. 13

  2. Isolation of Mitochondria from Mouse Skeletal Muscle for Respirometric Assays

    Journal of Visualized Experiments, Vol. 2022, Núm. 180

2021

  1. Coenzyme q at the hinge of health and metabolic diseases

    Antioxidants, Vol. 10, Núm. 11

  2. Model cells and organisms in mitochondrial diseases

    Mitochondrial Diseases: Theory, Diagnosis and Therapy (Springer International Publishing), pp. 231-271

  3. Secondary CoQ10 deficiency, bioenergetics unbalance in disease and aging

    BioFactors, Vol. 47, Núm. 4, pp. 551-569

2020

  1. Age-related mitochondrial dysfunction as a key factor in COVID-19 disease

    Experimental Gerontology, Vol. 142

  2. Coenzyme Q and Aging in the Fruit Fly Drosophila melanogaster

    Coenzyme Q in Aging (Springer International Publishing), pp. 141-155

2019

  1. ADCK2 haploinsufficiency reduces mitochondrial lipid oxidation and causes myopathy associated with CoQ deficiency

    Journal of Clinical Medicine, Vol. 8, Núm. 9

  2. 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

  3. 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

  1. Mitochondrial dysfunction in metabolism and ageing: shared mechanisms and outcomes?

    Biogerontology, Vol. 19, Núm. 6, pp. 461-480

  2. The mitochondrial phosphatase PPTC7 orchestrates mitochondrial metabolism regulating coenzyme Q10 biosynthesis

    Biochimica et Biophysica Acta - Bioenergetics, Vol. 1859, Núm. 11, pp. 1235-1248

2017

  1. Biochemical assessment of coenzyme Q10 deficiency

    Journal of Clinical Medicine, Vol. 6, Núm. 3

  2. 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

  3. Role of mitochondrial reverse electron transport in ROS signaling: Potential roles in health and disease

    Frontiers in Physiology, Vol. 8, Núm. JUN

2016

  1. Mitochondrial ROS Produced via Reverse Electron Transport Extend Animal Lifespan

    Cell Metabolism, Vol. 23, Núm. 4, pp. 725-734

  2. 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