Choose an application

• Reference Manager
• EndNote
• RefWorks (Direct export to RefWorks)

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.

Research & information: general --- Biology, life sciences --- Biochemistry --- aging heart --- Bcl-2 family --- mitochondria --- programmed cell death --- fatty acid oxidation --- palmitate --- oleate --- m.3243A&gt --- G mutation --- MT-ATP6 --- m.8909T&gt --- 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

Choose an application

• Reference Manager
• EndNote
• RefWorks (Direct export to RefWorks)

Mitochondria are subcellular organelles evolved by the endosymbiosis of bacteria with eukaryotic cells. They are the main source of ATP in the cell and engaged in other aspects of cell metabolism and cell function, including the regulation of ion homeostasis, cell growth, redox status, and cell signaling. Due to their central role in cell life and death, mitochondria are also involved in the pathogenesis and progression of human diseases/conditions, including neurodegenerative and cardiovascular disorders, cancer, diabetes, inflammation, and aging. However, despite the increasing number of studies, precise mechanisms whereby mitochondria are involved in the regulation of basic physiological functions, as well as their role in the cell under pathophysiological conditions, remain unknown. A lack of in-depth knowledge of the regulatory mechanisms of mitochondrial metabolism and function, as well as interplay between the factors that transform the organelle from its role in pro-survival to pro-death, have hindered the development of new mitochondria-targeted pharmacological and conditional approaches for the treatment of human diseases. This book highlights the latest achievements in elucidating the role of mitochondria under physiological conditions, in various cell/animal models of human diseases, and in patients.

Medicine --- hypoglycemia --- sodium dichloroacetate --- pyruvate dehydrogenase kinase --- pyruvate dehydrogenase --- oxidative stress --- neuron death --- cholangiocellular carcinoma --- mitochondria --- energy metabolism --- oxidative phosphorylation --- 4-HNE --- DRP1 --- ERK1/2 --- hippocampus --- JNK --- mitochondrial dynamics --- PKA --- protein phosphatases --- TUNEL --- DDE --- high-fat diet --- mitochondrial UCP2 --- ROS --- antioxidant system --- uncoupling protein --- mitochondria: energy metabolism --- lipid handling --- fatty acid oxidation --- potassium channel --- reactive oxygen species --- antioxidants --- life span --- aging --- BKCa channels --- pravastatin --- gemfibrozil --- liver --- colon --- mitochondrial function --- cyclosporin A --- mitochondria calcium buffering --- mitochondria bioenergetics --- mitochondria permeability transition pore --- inorganic phosphate --- hepatic fibrogenesis --- HtrA2/Omi --- reactive oxygen species stress --- mitochondrial homeostasis --- complex I (CI) deficiency --- metabolome and proteome profiling --- reactive oxygen species (ROS) --- respirasome assembly --- electron tunneling (ET) --- perilipin 5 --- lipid droplet --- H9c2 cardiomyoblasts --- adenine nucleotide translocase --- respiratory supercomplexes --- ETC complexes --- dentate granule cell --- epilepsy --- hyperforin --- LONP1 --- neuroprotection --- pilocarpine --- seizure --- siRNA --- cardioprotection --- mitochondrial permeability transition pores --- mitochondrial connexin 43 --- cardiolipin --- iron overload --- hepcidin --- transferrin --- ferritin --- ZIP --- inflammation --- mtDNA --- mitochondrial dysfunction --- muscle aging --- physical performance --- LHON --- Siberian population --- ancient mutation --- specific genetic background --- apoptosis --- human amniotic membrane --- mitochondrial cell death --- BAX --- BCL-2 --- tensile strength --- mitochondrial gene expression --- mtDNA transcription --- mtRNA --- post-transcriptional mtRNA processing --- dsRNA --- innate immunity --- interferon response --- amino acid neurotransmitter --- cerebellar amino acid metabolism --- hypoxia --- 2-oxoglutarate dehydrogenase --- tricarboxylic acid cycle --- heart --- cytoskeletal proteins --- mitochondrial interactions --- plectin --- tubulin beta --- signaling --- GW9662 --- ischemia reperfusion injury --- Langendorff --- myocardial --- pioglitazone --- redox state --- rosiglitazone --- TZD --- uncoupling --- ADP/ATP carrier --- KmADP --- dextran --- morphology --- cardiomyocytes --- telomere length --- telomerase activity --- development --- regeneration --- intranuclear mitochondria --- healthy cells --- electron and confocal microscopy --- signaling pathways --- ion homeostasis --- human diseases