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Mitochondrial Supercomplexes: Physiological Organization and Dysregulation in Age-Related Neurodegenerative Disorders
(Frontiers Media S.A., 2020-09)
Several studies suggest that the assembly of mitochondrial respiratory complexes into structures known as supercomplexes (SCs) may increase the efficiency of the electron transport chain, reducing the rate of production ...
Mitochondrial genotype modulates mtDNA copy number and organismal phenotype in Drosophila
(Elsevier B.V., 2017-05-01)
We evaluated the role of natural mitochondrial DNA (mtDNA) variation on mtDNA copy number, biochemical features and life history traits in Drosophila cybrid strains. We demonstrate the effects of both coding region and ...
Mitochondrial cytochrome c oxidase biogenesis: Recent developments
(Academic Press Ltd - Elsevier Science Ltd, 2018-04)
Mitochondrial cytochrome c oxidase (COX) is the primary site of cellular oxygen consumption and is essential for aerobic energy generation in the form of ATP. Human COX is a copper-heme A hetero-multimeric complex formed ...
Age-related decline in mitochondrial bioenergetics: Does supercomplex destabilization determine lower oxidative capacity and higher superoxide production
(Copyright, 2012-09-01)
Mitochondrial decay plays a central role in the aging process. Although certainly multifactorial in nature, defective operation of the electron transport chain (ETC) constitutes a key mechanism involved in the age-associated ...
Supercomplexes of the mitochondrial electron transport chain decline in the aging rat heart
(ELSEVIER SCIENCE INC, 2009-10-01)
Accumulation of mitochondrial electron transport chain (ETC) defects is a recognized hallmark of the age-associated decline in cardiac bioenergetics; however, the molecular events involved are only poorly understood. In ...
Mecanismos de produção de espécies reativas de oxigênio pelos complexos proteicos da cadeia transportadora de elétrons mitocondrial
(Universidade Federal de São Paulo, 2021-12-14)
Espécies reativas de oxigênio (ERO) são moléculas químicas derivadas do O2 que são mais reativas que o mesmo. A cadeia transportadora de elétrons, localizada na mitocôndria, é uma das principais fontes de produção dessas ...
Cyclophilin D over-expression increases mitochondrial complex III activity and accelerates supercomplex formation
(Elsevier Science Inc, 2017-01)
Cyclophilin D (CyPD), a mitochondrial matrix protein, has been widely studied for its role in mitochondrial-mediated cell death. Unexpectedly, we previously discovered that overexpression of CyPD in a stable cell line, ...
Frataxin Structure and Function
(Springer, 2019)
Mammalian frataxin is a small mitochondrial protein involved in iron sulfur cluster assembly. Frataxin deficiency causes the neurodegenerative disease Friedreich´s Ataxia. Valuable knowledge has been gained on the structural ...