Impaired Mitochondrial Bioenergetics under Pathological Conditions
Mitochondria are the powerhouses of cells; however, mitochondrial dysfunction causes energy depletion and cell death in a variety of diseases. Altered oxidative phosphorylation and ion homeostasis are associated with ROS production resulting from the disassembly of respiratory supercomplexes and the...
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| Language: | English |
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MDPI - Multidisciplinary Digital Publishing Institute
2022
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| Online Access: | ONIX_20220812_9783036546483_62 |
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| description | Mitochondria are the powerhouses of cells; however, mitochondrial dysfunction causes energy depletion and cell death in a variety of diseases. Altered oxidative phosphorylation and ion homeostasis are associated with ROS production resulting from the disassembly of respiratory supercomplexes and the disruption of electron transfer chains. In pathological conditions, the dysregulation of mitochondrial homeostasis promotes Ca2+ overload in the matrix and ROS accumulation, which induces the mitochondrial permeability transition pore formation responsible for mitochondrial morphological changes linked to membrane dynamics, and ultimately, cell death. Finally, studies on the impaired mitochondrial bioenergetics in pathology could provide molecular tools to counteract diseases associated with mitochondrial dysfunction. |
| format | Online |
| id | doab-20.500.12854ir-91183 |
| institution | Directory of Open Access Books |
| language | eng |
| publishDate | 2022 |
| publishDateRange | 2022 |
| publishDateSort | 2022 |
| publisher | MDPI - Multidisciplinary Digital Publishing Institute |
| publisherStr | MDPI - Multidisciplinary Digital Publishing Institute |
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| spelling | doab-20.500.12854ir-911832024-03-28T03:31:13Z Impaired Mitochondrial Bioenergetics under Pathological Conditions Lenaz, Giorgio Nesci, Salvatore aging heart Bcl-2 family mitochondria programmed cell death fatty acid oxidation palmitate oleate m.3243A> G mutation MT-ATP6 m.8909T> C ATP synthase nephropathy oxidative phosphorylation mitochondrial disease cardiolipin Barth syndrome Sengers syndrome respiratory chain Dilated Cardiomyopathy with Ataxia cardiomyopathy mammalian complex I NADH dehydrogenase complex I assembly complex I structure complex I deficiency supernumerary subunits electron transport chain mitochondrial dysfunction Leigh syndrome mitochondrial diseases yeast Saccharomyces cerevisiae pet mutants pancreatic endocrine cells mathematical model cellular bioenergetics diabetes glucagon insulin exercise immune system metabolic disease COVID-19 mitochondrial dynamics viral infections MAVS RIG-I MDA5 innate immune response SARS CoV-2 RSV influenza respiratory supercomplexes ROS ATP synthase/hydrolase mitochondrial permeability transition pore cristae cellular signaling human disease mitochondrial dynamic cell signaling cancer respiratory complexes oxidative stress mitochondrial DNA MTCYB mutations cytochrome b complex III aging energy metabolism entorhinal cortex lipoxidation-derived damage neurodegeneration oxidative damage protein import respiratory complex assembly supercomplexes mitochondrial proteostasis heart failure bioenergetics assembly factor atypical myopathy high-resolution respirometry toxicity assays cell culture equine primary myoblasts fibroblasts frozen tissue leukocytes oxygen consumption platelets respirometry skeletal muscle n/a thema EDItEUR::G Reference, Information and Interdisciplinary subjects::GP Research and information: general thema EDItEUR::P Mathematics and Science::PS Biology, life sciences thema EDItEUR::P Mathematics and Science::PS Biology, life sciences::PSB Biochemistry Mitochondria are the powerhouses of cells; however, mitochondrial dysfunction causes energy depletion and cell death in a variety of diseases. Altered oxidative phosphorylation and ion homeostasis are associated with ROS production resulting from the disassembly of respiratory supercomplexes and the disruption of electron transfer chains. In pathological conditions, the dysregulation of mitochondrial homeostasis promotes Ca2+ overload in the matrix and ROS accumulation, which induces the mitochondrial permeability transition pore formation responsible for mitochondrial morphological changes linked to membrane dynamics, and ultimately, cell death. Finally, studies on the impaired mitochondrial bioenergetics in pathology could provide molecular tools to counteract diseases associated with mitochondrial dysfunction. 2022-08-12T12:48:36Z 2022-08-12T12:48:36Z 2022 book ONIX_20220812_9783036546483_62 9783036546483 9783036546476 https://directory.doabooks.org/handle/20.500.12854/91183 eng image/jpeg Attribution 4.0 International https://mdpi.com/books/pdfview/book/5829 MDPI - Multidisciplinary Digital Publishing Institute 10.3390/books978-3-0365-4647-6 10.3390/books978-3-0365-4647-6 46cabcaa-dd94-4bfe-87b4-55023c1b36d0 9783036546483 9783036546476 460 Basel open access |
| spellingShingle | aging heart Bcl-2 family mitochondria programmed cell death fatty acid oxidation palmitate oleate m.3243A> G mutation MT-ATP6 m.8909T> C ATP synthase nephropathy oxidative phosphorylation mitochondrial disease cardiolipin Barth syndrome Sengers syndrome respiratory chain Dilated Cardiomyopathy with Ataxia cardiomyopathy mammalian complex I NADH dehydrogenase complex I assembly complex I structure complex I deficiency supernumerary subunits electron transport chain mitochondrial dysfunction Leigh syndrome mitochondrial diseases yeast Saccharomyces cerevisiae pet mutants pancreatic endocrine cells mathematical model cellular bioenergetics diabetes glucagon insulin exercise immune system metabolic disease COVID-19 mitochondrial dynamics viral infections MAVS RIG-I MDA5 innate immune response SARS CoV-2 RSV influenza respiratory supercomplexes ROS ATP synthase/hydrolase mitochondrial permeability transition pore cristae cellular signaling human disease mitochondrial dynamic cell signaling cancer respiratory complexes oxidative stress mitochondrial DNA MTCYB mutations cytochrome b complex III aging energy metabolism entorhinal cortex lipoxidation-derived damage neurodegeneration oxidative damage protein import respiratory complex assembly supercomplexes mitochondrial proteostasis heart failure bioenergetics assembly factor atypical myopathy high-resolution respirometry toxicity assays cell culture equine primary myoblasts fibroblasts frozen tissue leukocytes oxygen consumption platelets respirometry skeletal muscle n/a thema EDItEUR::G Reference, Information and Interdisciplinary subjects::GP Research and information: general thema EDItEUR::P Mathematics and Science::PS Biology, life sciences thema EDItEUR::P Mathematics and Science::PS Biology, life sciences::PSB Biochemistry Impaired Mitochondrial Bioenergetics under Pathological Conditions |
| title | Impaired Mitochondrial Bioenergetics under Pathological Conditions |
| title_full | Impaired Mitochondrial Bioenergetics under Pathological Conditions |
| title_fullStr | Impaired Mitochondrial Bioenergetics under Pathological Conditions |
| title_full_unstemmed | Impaired Mitochondrial Bioenergetics under Pathological Conditions |
| title_short | Impaired Mitochondrial Bioenergetics under Pathological Conditions |
| title_sort | impaired mitochondrial bioenergetics under pathological conditions |
| topic | aging heart Bcl-2 family mitochondria programmed cell death fatty acid oxidation palmitate oleate m.3243A> G mutation MT-ATP6 m.8909T> C ATP synthase nephropathy oxidative phosphorylation mitochondrial disease cardiolipin Barth syndrome Sengers syndrome respiratory chain Dilated Cardiomyopathy with Ataxia cardiomyopathy mammalian complex I NADH dehydrogenase complex I assembly complex I structure complex I deficiency supernumerary subunits electron transport chain mitochondrial dysfunction Leigh syndrome mitochondrial diseases yeast Saccharomyces cerevisiae pet mutants pancreatic endocrine cells mathematical model cellular bioenergetics diabetes glucagon insulin exercise immune system metabolic disease COVID-19 mitochondrial dynamics viral infections MAVS RIG-I MDA5 innate immune response SARS CoV-2 RSV influenza respiratory supercomplexes ROS ATP synthase/hydrolase mitochondrial permeability transition pore cristae cellular signaling human disease mitochondrial dynamic cell signaling cancer respiratory complexes oxidative stress mitochondrial DNA MTCYB mutations cytochrome b complex III aging energy metabolism entorhinal cortex lipoxidation-derived damage neurodegeneration oxidative damage protein import respiratory complex assembly supercomplexes mitochondrial proteostasis heart failure bioenergetics assembly factor atypical myopathy high-resolution respirometry toxicity assays cell culture equine primary myoblasts fibroblasts frozen tissue leukocytes oxygen consumption platelets respirometry skeletal muscle n/a thema EDItEUR::G Reference, Information and Interdisciplinary subjects::GP Research and information: general thema EDItEUR::P Mathematics and Science::PS Biology, life sciences thema EDItEUR::P Mathematics and Science::PS Biology, life sciences::PSB Biochemistry |
| topic_facet | aging heart Bcl-2 family mitochondria programmed cell death fatty acid oxidation palmitate oleate m.3243A> G mutation MT-ATP6 m.8909T> C ATP synthase nephropathy oxidative phosphorylation mitochondrial disease cardiolipin Barth syndrome Sengers syndrome respiratory chain Dilated Cardiomyopathy with Ataxia cardiomyopathy mammalian complex I NADH dehydrogenase complex I assembly complex I structure complex I deficiency supernumerary subunits electron transport chain mitochondrial dysfunction Leigh syndrome mitochondrial diseases yeast Saccharomyces cerevisiae pet mutants pancreatic endocrine cells mathematical model cellular bioenergetics diabetes glucagon insulin exercise immune system metabolic disease COVID-19 mitochondrial dynamics viral infections MAVS RIG-I MDA5 innate immune response SARS CoV-2 RSV influenza respiratory supercomplexes ROS ATP synthase/hydrolase mitochondrial permeability transition pore cristae cellular signaling human disease mitochondrial dynamic cell signaling cancer respiratory complexes oxidative stress mitochondrial DNA MTCYB mutations cytochrome b complex III aging energy metabolism entorhinal cortex lipoxidation-derived damage neurodegeneration oxidative damage protein import respiratory complex assembly supercomplexes mitochondrial proteostasis heart failure bioenergetics assembly factor atypical myopathy high-resolution respirometry toxicity assays cell culture equine primary myoblasts fibroblasts frozen tissue leukocytes oxygen consumption platelets respirometry skeletal muscle n/a thema EDItEUR::G Reference, Information and Interdisciplinary subjects::GP Research and information: general thema EDItEUR::P Mathematics and Science::PS Biology, life sciences thema EDItEUR::P Mathematics and Science::PS Biology, life sciences::PSB Biochemistry |
| url | ONIX_20220812_9783036546483_62 |