ArticleMolecular neurodegeneration2017
Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo.
Article in Molecular neurodegeneration, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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29 citing papers in PubMed, 40 citations in OpenAlex.
- Therapeutic targeting of the conserved region within the low-complexity domain of TDP-43 is neuroprotective and extends survival in amyotrophic lateral sclerosis mice.Nature aging · 2026Article
- TDP-43: [GU]-ardian of the transcriptome.Molecular neurodegeneration · 2026Review
- The mechanisms underlying TDP-43-associated neurodegeneration in Alzheimer's disease and related dementias.Molecular psychiatry · 2025Review
- Sex-Specific Early Retinal Dysfunction in Mutant TDP-43 Transgenic Mice.Journal of Alzheimer's disease : JAD · 2024Article
- Nutritional and metabolic factors in amyotrophic lateral sclerosis.Nature reviews. Neurology · 2023Review
- Altered TDP-43 Structure and Function: Key Insights into Aberrant RNA, Mitochondrial, and Cellular and Systemic Metabolism in Amyotrophic Lateral Sclerosis.Metabolites · 2022Review
- Regulation of Endoplasmic Reticulum-Mitochondria Tethering and CaInternational journal of molecular sciences · 2021Article
- Multi-phaseted problems of TDP-43 in selective neuronal vulnerability in ALS.Cellular and molecular life sciences : CMLS · 2021Review
- Mitochondrion-Dependent Cell Death in TDP-43 Proteinopathies.Biomedicines · 2021Review
- The Interplay of RNA Binding Proteins, Oxidative Stress and Mitochondrial Dysfunction in ALS.Antioxidants (Basel, Switzerland) · 2021Review
- Maintaining the balance of TDP-43, mitochondria, and autophagy: a promising therapeutic strategy for neurodegenerative diseases.Translational neurodegeneration · 2020Review
- The overexpression of TDP-43 in astrocytes causes neurodegeneration via a PTP1B-mediated inflammatory response.Journal of neuroinflammation · 2020Article
- Accelerated transsulfuration metabolically defines a discrete subclass of amyotrophic lateral sclerosis patients.Neurobiology of disease · 2020Article
- The role of TDP-43 mislocalization in amyotrophic lateral sclerosis.Molecular neurodegeneration · 2020Review
- Review
- Emerging Evidence Highlighting the Importance of Redox Dysregulation in the Pathogenesis of Amyotrophic Lateral Sclerosis (ALS).Frontiers in cellular neuroscience · 2020Article
- Current and Emerging Approaches for Studying Inter-Organelle Membrane Contact Sites.Frontiers in cell and developmental biology · 2020Review
- TDP-43 inhibitory peptide alleviates neurodegeneration and memory loss in an APP transgenic mouse model for Alzheimer's disease.Biochimica et biophysica acta. Molecular basis of disease · 2020Article
- TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration.Molecular and cellular neurosciences · 2019Review
- Association between TDP-43 and mitochondria in inclusion body myositis.Laboratory investigation; a journal of technical methods and pathology · 2019Article
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
Abstract
backgroundMitochondrial dysfunction has been linked to the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Functional studies of mitochondrial bioenergetics have focused mostly on superoxide dismutase 1 (SOD1) mutants, and showed that mutant human SOD1 impairs mitochondrial oxidative phosphorylation, calcium homeostasis, and dynamics. However, recent reports have indicated that alterations in transactivation response element DNA-binding protein 43 (TDP-43) can also lead to defects of mitochondrial morphology and dynamics. Furthermore, it was proposed that TDP-43 mutations cause oxidative phosphorylation impairment associated with respiratory chain defects and that these effects were caused by mitochondrial localization of the mutant protein. Here, we investigated the presence of bioenergetic defects in the brain of transgenic mice expressing human mutant TDP-43 (TDP-43
methodsIn the brain of TDP-43
resultsWe did not observe defects in any of the mitochondrial bioenergetic functions that were tested in TDP-43 mutants. We detected a small amount of TDP-43
conclusionsWhile alterations of mitochondrial morphology and dynamics in TDP-43 mutant neurons are well established, the present study did not demonstrate oxidative phosphorylation defects in TDP-43 mutants, in vitro and in vivo. On the other hand, the increase in mitochondrial calcium uptake in A315T TDP-43 mutants was an intriguing finding, which needs to be investigated further to understand its mechanisms and potential pathogenic implications.
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