ArticleMetabolomics : Official journal of the Metabolomic Society2018
NMR metabolomics of fibroblasts with inherited mitochondrial Complex I mutation reveals treatment-reversible lipid and amino acid metabolism alterations.
Article in Metabolomics : Official journal of the Metabolomic Society, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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16 citing papers in PubMed, 21 citations in OpenAlex.
- Decoding rare inherited metabolic disorders: advancing precision in screening and diagnosis.Orphanet journal of rare diseases · 2026Review
- Intra- and extracellular real-time analysis of perfused fibroblasts using an NMR bioreactor: A pilot study.Journal of inherited metabolic disease · 2025Article
- Chemotherapy-Induced Peripheral Neuropathy.Handbook of experimental pharmacology · 2023Article
- Mitochondrial Complex I Disruption Causes Broad Reorchestration of Plant Lipidome Including Chloroplast Lipids.International journal of molecular sciences · 2022Article
- Article
- Determination of Intra- and Extracellular Metabolic Adaptations of 3D Cell Cultures upon Challenges in Real-Time by NMR.International journal of molecular sciences · 2022Article
- The Radioprotective Activity of Resveratrol-Metabolomic Point of View.Metabolites · 2022Review
- Metabolic Regulation of Fibroblast Activation and Proliferation during Organ Fibrosis.Kidney diseases (Basel, Switzerland) · 2022Review
- A SERS Platform for Rapid Detection of Drug Resistance of Non-International journal of nanomedicine · 2022Article
- Multi-mtDNA Variants May Be a Factor Contributing to Mitochondrial Function Variety in the Skin-Derived Fibroblasts of Leber's Hereditary Optic Neuropathy Patients.Frontiers in molecular neuroscience · 2022Article
- Superoxide dismutase 2 ameliorates mitochondrial dysfunction in skin fibroblasts of Leber's hereditary optic neuropathy patients.Frontiers in neuroscience · 2022Article
- A plasma metabolomic signature of Leber hereditary optic neuropathy showing taurine and nicotinamide deficiencies.Human molecular genetics · 2021Article
- Therapeutic Approaches to Treat Mitochondrial Diseases: "One-Size-Fits-All" and "Precision Medicine" Strategies.Pharmaceutics · 2020Review
- Metabolomic study on bleomycin and polyhexamethylene guanidine phosphate-induced pulmonary fibrosis mice models.Metabolomics : Official journal of the Metabolomic Society · 2019Article
- Review
- Selective galactose culture condition reveals distinct metabolic signatures in pyruvate dehydrogenase and complex I deficient human skin fibroblasts.Metabolomics : Official journal of the Metabolomic Society · 2019Article
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2 authors at 1 institution in 1 country.
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Abstract
introductionElucidating molecular alterations due to mitochondrial Complex I (CI) mutations may help to understand CI deficiency (CID), not only in mitochondriopathies but also as it is caused by drugs or associated to many diseases.
objectivesCID metabolic expression was investigated in Leber's hereditary optic neuropathy (LHON) caused by an inherited mutation of CI.
methodsNMR-based metabolomics analysis was performed in intact skin fibroblasts from LHON patients. It used several datasets: one-dimensional
resultsLHON fibroblasts had decreased CI activity (- 43%, p < 0.01). Metabolomics revealed prominent alterations in LHON including the increase of fatty acids (FA), polyunsaturated FA and phosphatidylcholine with a variable importance in the prediction (VIP) > 1 in OPLS-DA, p < 0.01 in univariate statistics, and the decrease of amino acids (AA), predominantly glycine, glutamate, glutamine (VIP > 1) and alanine (VIP > 1, p < 0.05). In LHON, treatment with IDE and RSV increased CI activity (+ 40 and + 44%, p < 0.05). IDE decreased FA, polyunsaturated FA and phosphatidylcholine (p < 0.05), but did not modified AA levels. RSV decreased polyunsaturated FA, and increased several AA (VIP > 1 and/or p < 0.05).
conclusionLHON fibroblasts display lipid and amino acid metabolism alterations that are reversed by mitochondria-targeted treatments, and can be related to adaptive changes. Findings bring insights into molecular changes induced by CI mutation and, beyond, CID of other origins.
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