ArticleHuman molecular genetics2014
Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.
Article in Human molecular genetics, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 42 citations in OpenAlex.
- NDUFA5 deficiency promotes renal inflammation in diabetic nephropathy via mitochondrial ROS signaling.iScience · 2026Article
- Bi-allelic variants in NDUFA5 cause a mitochondriopathy with complex I deficiency.American journal of human genetics · 2026Article
- Intestinal epithelial Dicer1 regulates gut microbiome and Alzheimer's pathology in App-knock-in mice.Alzheimer's research & therapy · 2025Article
- Deficient AMPK-SENP1-Sirt3 signaling impairs mitochondrial complex I function in Parkinson's disease model.Translational neurodegeneration · 2025Article
- A Comprehensive Review of Mitochondrial Complex I During Mammalian Oocyte Maturation.Genesis (New York, N.Y. : 2000) · 2025Review
- Target engagement of ginsenosides in mild cognitive impairment using mass spectrometry-based drug affinity responsive target stability.Journal of ginseng research · 2022Article
- Differential requirements for mitochondrial electron transport chain components in the adult murine liver.eLife · 2022Article
- Chlamydia pneumoniae can infect the central nervous system via the olfactory and trigeminal nerves and contributes to Alzheimer's disease risk.Scientific reports · 2022Article
- Deficits in Prenatal Serine Biosynthesis Underlie the Mitochondrial Dysfunction Associated with the Autism-LinkedInternational journal of molecular sciences · 2021Article
- The Mysterious Multitude: Structural Perspective on the Accessory Subunits of Respiratory Complex I.Frontiers in molecular biosciences · 2021Review
- Adult mesenchymal stem cell ageing interplays with depressed mitochondrial Ndufs6.Cell death & disease · 2020Article
- Metformin delays neurological symptom onset in a mouse model of neuronal complex I deficiency.JCI insight · 2020Article
- Proteomics Reveals the Potential Protective Mechanism of Hydrogen Sulfide on Retinal Ganglion Cells in an Ischemia/Reperfusion Injury Animal Model.Pharmaceuticals (Basel, Switzerland) · 2020Article
- ALS/FTD-associated protein FUS induces mitochondrial dysfunction by preferentially sequestering respiratory chain complex mRNAs.Genes & development · 2020Article
- Mitochondria dysfunction in the pathogenesis of Alzheimer's disease: recent advances.Molecular neurodegeneration · 2020Review
- Prediction of tumor location in prostate cancer tissue using a machine learning system on gene expression data.BMC bioinformatics · 2020Article
- Myopathy reversion in mice after restauration of mitochondrial complex I.EMBO molecular medicine · 2020Article
- iTRAQ-Based Proteomic Analysis of Dentate Gyrus in Temporal Lobe Epilepsy With Hippocampal Sclerosis.Frontiers in neurology · 2020Article
- Systematic analysis of microarray datasets to identify Parkinson's disease‑associated pathways and genes.Molecular medicine reports · 2017Article
- Review: Central nervous system involvement in mitochondrial disease.Neuropathology and applied neurobiology · 2017Review
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
Deficiencies in the complex I (CI; NADH-ubiquinone oxidoreductase) of the respiratory chain are frequent causes of mitochondrial diseases and have been associated with other neurodegenerative disorders, such as Parkinson's disease. The NADH-ubiquinone oxidoreductase 1 alpha subcomplex subunit 5 (NDUFA5) is a nuclear-encoded structural subunit of CI, located in the peripheral arm. We inactivated Ndufa5 in mice by the gene-trap methodology and found that this protein is required for embryonic survival. Therefore, we have created a conditional Ndufa5 knockout (KO) allele by introducing a rescuing Ndufa5 cDNA transgene flanked by loxP sites, which was selectively ablated in neurons by the CaMKIIα-Cre. At the age of 11 months, mice with a central nervous system knockout of Ndufa5 (Ndufa5 CNS-KO) showed lethargy and loss of motor skills. In these mice cortices, the levels of NDUFA5 protein were reduced to 25% of controls. Fully assembled CI levels were also greatly reduced in cortex and CI activity in homogenates was reduced to 60% of controls. Despite the biochemical phenotype, no oxidative damage, neuronal death or gliosis were detected in the Ndufa5 CNS-KO brain at this age. These results showed that a partial defect in CI in neurons can lead to late-onset motor phenotypes without neuronal loss or oxidative damage.
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