Publications (24) Paula Cilleros Holgado publications

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2025

  1. Alpha–lipoic acid supplementation improves pathological alterations in cellular models of Friedreich ataxia

    Orphanet Journal of Rare Diseases , Vol. 20, Núm. 1

  2. Biotin Induces Inactive Chromosome X Reactivation and Corrects Physiopathological Alterations in Beta-Propeller-Protein-Associated Neurodegeneration

    International Journal of Molecular Sciences, Vol. 26, Núm. 3

  3. Iron Accumulation and Lipid Peroxidation in Cellular Models of Nemaline Myopathies

    International Journal of Molecular Sciences, Vol. 26, Núm. 4

  4. Mitochondrial Unfolded Protein Response (mtUPR) Activation Improves Pathological Alterations in Cellular Models of Ethylmalonic Encephalopathy

    Antioxidants, Vol. 14, Núm. 6

  5. Mitochondrial dysfunction, iron accumulation, lipid peroxidation, and inflammasome activation in cellular models derived from patients with multiple sclerosis

    Aging, Vol. 17, Núm. 2, pp. 365-392

  6. Pathophysiology, biological models and new therapeutic approaches in β-Propeller Associated Neurodegeneration

    International Review of Cell and Molecular Biology (Elsevier Inc.)

  7. Polydatin and Nicotinamide Prevent Iron Accumulation and Lipid Peroxidation in Cellular Models of Mitochondrial Diseases

    Antioxidants, Vol. 14, Núm. 2

2024

  1. A Multi-Target Pharmacological Correction of a Lipoyltransferase LIPT1 Gene Mutation in Patient-Derived Cellular Models

    Antioxidants, Vol. 13, Núm. 8

  2. Polydatin and Nicotinamide Rescue the Cellular Phenotype of Mitochondrial Diseases by Mitochondrial Unfolded Protein Response (mtUPR) Activation

    Biomolecules, Vol. 14, Núm. 5

2023

  1. Actin Polymerization Defects Induce Mitochondrial Dysfunction in Cellular Models of Nemaline Myopathies

    Antioxidants, Vol. 12, Núm. 12

  2. Alpha-lipoic acid supplementation corrects pathological alterations in cellular models of pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels

    Orphanet journal of rare diseases, Vol. 18, Núm. 1, pp. 80

  3. Antioxidants Prevent Iron Accumulation and Lipid Peroxidation, but Do Not Correct Autophagy Dysfunction or Mitochondrial Bioenergetics in Cellular Models of BPAN

    International Journal of Molecular Sciences, Vol. 24, Núm. 19

  4. Mitochondrial Quality Control via Mitochondrial Unfolded Protein Response (mtUPR) in Ageing and Neurodegenerative Diseases

    Biomolecules, Vol. 13, Núm. 12

  5. Neurodegeneration, Mitochondria, and Antibiotics

    Metabolites, Vol. 13, Núm. 3

  6. Patient-Derived Cellular Models for Polytarget Precision Medicine in Pantothenate Kinase-Associated Neurodegeneration

    Pharmaceuticals, Vol. 16, Núm. 10

  7. Vicious cycle of lipid peroxidation and iron accumulation in neurodegeneration

    Neural Regeneration Research, Vol. 18, Núm. 6, pp. 1196-1202

  8. mtUPR Modulation as a Therapeutic Target for Primary and Secondary Mitochondrial Diseases

    International Journal of Molecular Sciences, Vol. 24, Núm. 2

2022

  1. Activation of the Mitochondrial Unfolded Protein Response: A New Therapeutic Target?

    Biomedicines, Vol. 10, Núm. 7

  2. Pantothenate and L-Carnitine Supplementation Improves Pathological Alterations in Cellular Models of KAT6A Syndrome

    Genes, Vol. 13, Núm. 12

  3. Therapeutic approach with commercial supplements for pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels

    Orphanet journal of rare diseases, Vol. 17, Núm. 1, pp. 311