05197nam 2201537z- 450 991058593450332120231214133056.0(CKB)5600000000483143(oapen)https://directory.doabooks.org/handle/20.500.12854/91183(EXLCZ)99560000000048314320202208d2022 |y 0engurmn|---annantxtrdacontentcrdamediacrrdacarrierImpaired Mitochondrial Bioenergetics under Pathological ConditionsBaselMDPI - Multidisciplinary Digital Publishing Institute20221 electronic resource (460 p.)3-0365-4648-0 3-0365-4647-2 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: generalbicsscBiology, life sciencesbicsscBiochemistrybicsscaging heartBcl-2 familymitochondriaprogrammed cell deathfatty acid oxidationpalmitateoleatem.3243A&gtG mutationMT-ATP6m.8909T&gtCATP synthasenephropathyoxidative phosphorylationmitochondrial diseasecardiolipinBarth syndromeSengers syndromerespiratory chainDilated Cardiomyopathy with Ataxiacardiomyopathymammalian complex INADH dehydrogenasecomplex I assemblycomplex I structurecomplex I deficiencysupernumerary subunitselectron transport chainmitochondrial dysfunctionLeigh syndromemitochondrial diseasesyeastSaccharomyces cerevisiaepet mutantspancreatic endocrine cellsmathematical modelcellular bioenergeticsdiabetesglucagoninsulinexerciseimmune systemmetabolic diseaseCOVID-19mitochondrial dynamicsviral infectionsMAVSRIG-IMDA5innate immune responseSARS CoV-2RSVinfluenzarespiratory supercomplexesROSATP synthase/hydrolasemitochondrial permeability transition porecristaecellular signalinghuman diseasemitochondrial dynamiccell signalingcancerrespiratory complexesoxidative stressmitochondrial DNAMTCYB mutationscytochrome bcomplex IIIagingenergy metabolismentorhinal cortexlipoxidation-derived damageneurodegenerationoxidative damageprotein importrespiratory complex assemblysupercomplexesmitochondrial proteostasisheart failurebioenergeticsassembly factoratypical myopathyhigh-resolution respirometrytoxicity assayscell cultureequine primary myoblastsfibroblastsfrozen tissueleukocytesoxygen consumptionplateletsrespirometryskeletal muscleResearch & information: generalBiology, life sciencesBiochemistryLenaz Giorgioedt100854Nesci SalvatoreedtLenaz GiorgioothNesci SalvatoreothBOOK9910585934503321Impaired Mitochondrial Bioenergetics under Pathological Conditions3035973UNINA