Mitochondrial disorders of the OXPHOS system
E Fernandez‐Vizarra, M Zeviani - FEBS letters, 2021 - Wiley Online Library
Mitochondrial disorders are among the most frequent inborn errors of metabolism, their
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …
primary cause being the dysfunction of the oxidative phosphorylation system (OXPHOS) …
The genetics and pathology of mitochondrial disease
Mitochondria are double‐membrane‐bound organelles that are present in all nucleated
eukaryotic cells and are responsible for the production of cellular energy in the form of ATP …
eukaryotic cells and are responsible for the production of cellular energy in the form of ATP …
Accessory subunits are integral for assembly and function of human mitochondrial complex I
DA Stroud, EE Surgenor, LE Formosa, B Reljic… - Nature, 2016 - nature.com
Complex I (NADH: ubiquinone oxidoreductase) is the first enzyme of the mitochondrial
respiratory chain and is composed of 45 subunits in humans, making it one of the largest …
respiratory chain and is composed of 45 subunits in humans, making it one of the largest …
Mitochondrial structure and bioenergetics in normal and disease conditions
M Protasoni, M Zeviani - International journal of molecular sciences, 2021 - mdpi.com
Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and
involved in various aspects of cellular life, with a primary role in energy production. The …
involved in various aspects of cellular life, with a primary role in energy production. The …
Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes
A Signes, E Fernandez-Vizarra - Essays in biochemistry, 2018 - portlandpress.com
The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …
inner mitochondrial membrane is an intricate process. The human enzymes comprise core …
[HTML][HTML] MLKL, the protein that mediates necroptosis, also regulates endosomal trafficking and extracellular vesicle generation
Activation of the pseudokinase mixed lineage kinase domain-like (MLKL) upon its
phosphorylation by the protein kinase RIPK3 triggers necroptosis, a form of programmed cell …
phosphorylation by the protein kinase RIPK3 triggers necroptosis, a form of programmed cell …
MiD49 and MiD51, new components of the mitochondrial fission machinery
CS Palmer, LD Osellame, D Laine… - EMBO …, 2011 - embopress.org
Mitochondria form intricate networks through fission and fusion events. Here, we identify
mitochondrial dynamics proteins of 49 and 51 kDa (MiD49 and MiD51, respectively) …
mitochondrial dynamics proteins of 49 and 51 kDa (MiD49 and MiD51, respectively) …
[HTML][HTML] Understanding mitochondrial complex I assembly in health and disease
M Mimaki, X Wang, M McKenzie, DR Thorburn… - … et Biophysica Acta (BBA …, 2012 - Elsevier
Complex I (NADH: ubiquinone oxidoreductase) is the largest multimeric enzyme complex of
the mitochondrial respiratory chain, which is responsible for electron transport and the …
the mitochondrial respiratory chain, which is responsible for electron transport and the …
Adaptor proteins MiD49 and MiD51 can act independently of Mff and Fis1 in Drp1 recruitment and are specific for mitochondrial fission
Drp1 (dynamin-related protein 1) is recruited to both mitochondrial and peroxisomal
membranes to execute fission. Fis1 and Mff are Drp1 receptor/effector proteins of …
membranes to execute fission. Fis1 and Mff are Drp1 receptor/effector proteins of …
Building a complex complex: Assembly of mitochondrial respiratory chain complex I
Mitochondrial complex I is the primary entry point for electrons into the electron transport
chain, required for the bulk of cellular ATP production via oxidative phosphorylation …
chain, required for the bulk of cellular ATP production via oxidative phosphorylation …