ArticleBiochimica et biophysica acta. Bioenergetics2017
Subunit NDUFV3 is present in two distinct isoforms in mammalian complex I.
Article in Biochimica et biophysica acta. Bioenergetics, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 42 citations in OpenAlex.
- Review
- NDUFB7 mutations cause brain neuronal defects, lactic acidosis, and mitochondrial dysfunction in humans and zebrafish.Cell death discovery · 2025Article
- Proton-Translocating NADH-Ubiquinone Oxidoreductase: Interaction with Artificial Electron Acceptors, Inhibitors, and Potential Medicines.International journal of molecular sciences · 2024Review
- Cellular senescence induced by down-regulation ofLife medicine · 2024Article
- Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4The EMBO journal · 2024Article
- Resting mitochondrial complex I fromeLife · 2023Article
- Conformational changes in mitochondrial complex I of the thermophilic eukaryoteScience advances · 2022Article
- Membrane-domain mutations in respiratory complex I impede catalysis but do not uncouple proton pumping from ubiquinone reduction.PNAS nexus · 2022Article
- YbeY is required for ribosome small subunit assembly and tRNA processing in human mitochondria.Nucleic acids research · 2021Article
- Accessory Subunits of the Matrix Arm of Mitochondrial Complex I with a Focus on Subunit NDUFS4 and Its Role in Complex I Function and Assembly.Life (Basel, Switzerland) · 2021Review
- Regulation of ATP hydrolysis by the ε subunit, ζ subunit and Mg-ADP in the ATP synthase of Paracoccus denitrificans.Biochimica et biophysica acta. Bioenergetics · 2021Article
- Complexome profile of Toxoplasma gondii mitochondria identifies divergent subunits of respiratory chain complexes including new subunits of cytochrome bc1 complex.PLoS pathogens · 2021Article
- The Mysterious Multitude: Structural Perspective on the Accessory Subunits of Respiratory Complex I.Frontiers in molecular biosciences · 2021Review
- Complexome Profiling-Exploring Mitochondrial Protein Complexes in Health and Disease.Frontiers in cell and developmental biology · 2021Review
- Analysis of Human Mutations in the Supernumerary Subunits of Complex I.Life (Basel, Switzerland) · 2020Review
- Mitochondrial complex I structure reveals ordered water molecules for catalysis and proton translocation.Nature structural & molecular biology · 2020Article
- Insights from Drosophila on mitochondrial complex I.Cellular and molecular life sciences : CMLS · 2020Review
- Splice-Junction-Based Mapping of Alternative Isoforms in the Human Proteome.Cell reports · 2019Article
- Human primitive brain displays negative mitochondrial-nuclear expression correlation of respiratory genes.Genome research · 2018Article
- Cryo-EM structures of complex I from mouse heart mitochondria in two biochemically defined states.Nature structural & molecular biology · 2018Article
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Authors and funding
4 authors at 1 institution in 1 country.
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Abstract
Complex I (NADH:ubiquinone oxidoreductase) is the first enzyme of the electron transport chain in mammalian mitochondria. Extensive proteomic and structural analyses of complex I from Bos taurus heart mitochondria have shown it comprises 45 subunits encoded on both the nuclear and mitochondrial genomes; 44 of them are different and one is present in two copies. The bovine heart enzyme has provided a model for studying the composition of complex I in other mammalian species, including humans, but the possibility of additional subunits or isoforms in other species or tissues has not been explored. Here, we describe characterization of the complexes I purified from five rat tissues and from a rat hepatoma cell line. We identify a~50kDa isoform of subunit NDUFV3, for which the canonical isoform is only ~10kDa in size. We combine LC-MS and MALDI-TOF mass spectrometry data from two different purification methods (chromatography and immuno-purification) with information from blue native PAGE analyses to show the long isoform is present in the mature complex, but at substoichiometric levels. It is also present in complex I in cultured human cells. We describe evidence that the long isoform is more abundant in both the mitochondria and purified complexes from brain (relative to in heart, liver, kidney and skeletal muscle) and more abundant still in complex I in cultured cells. We propose that the long 50kDa isoform competes with its canonical 10kDa counterpart for a common binding site on the flavoprotein domain of complex I.
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