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) …
Mechanisms of mitochondrial iron-sulfur protein biogenesis
R Lill, SA Freibert - Annual review of biochemistry, 2020 - annualreviews.org
Mitochondria are essential in most eukaryotes and are involved in numerous biological
functions including ATP production, cofactor biosyntheses, apoptosis, lipid synthesis, and …
functions including ATP production, cofactor biosyntheses, apoptosis, lipid synthesis, and …
[HTML][HTML] Mechanistic concepts of iron-sulfur protein biogenesis in Biology
JJ Braymer, SA Freibert, M Rakwalska-Bange… - Biochimica et Biophysica …, 2021 - Elsevier
Abstract Iron-sulfur (Fe/S) proteins are present in virtually all living organisms and are
involved in numerous cellular processes such as respiration, photosynthesis, metabolic …
involved in numerous cellular processes such as respiration, photosynthesis, metabolic …
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 …
Iron–sulfur cluster biogenesis and trafficking in mitochondria
JJ Braymer, R Lill - Journal of Biological Chemistry, 2017 - ASBMB
The biogenesis of iron–sulfur (Fe/S) proteins in eukaryotes is a multistage,
multicompartment process that is essential for a broad range of cellular functions, including …
multicompartment process that is essential for a broad range of cellular functions, including …
Function and biogenesis of iron–sulphur proteins
R Lill - Nature, 2009 - nature.com
Abstract Iron–sulphur (Fe–S) clusters have long been recognized as essential and versatile
cofactors of proteins involved in catalysis, electron transport and sensing of ambient …
cofactors of proteins involved in catalysis, electron transport and sensing of ambient …
[HTML][HTML] Mammalian iron metabolism and its control by iron regulatory proteins
CP Anderson, M Shen, RS Eisenstein… - Biochimica et Biophysica …, 2012 - Elsevier
Cellular iron homeostasis is maintained by iron regulatory proteins 1 and 2 (IRP1 and IRP2).
IRPs bind to iron-responsive elements (IREs) located in the untranslated regions of mRNAs …
IRPs bind to iron-responsive elements (IREs) located in the untranslated regions of mRNAs …
Mitochondrial respiratory chain complexes
Mitochondria are the power stations of the eukaryotic cell, using the energy released by the
oxidation of glucose and other sugars to produce ATP. Electrons are transferred from NADH …
oxidation of glucose and other sugars to produce ATP. Electrons are transferred from NADH …
[HTML][HTML] The role of mitochondria in cellular iron–sulfur protein biogenesis and iron metabolism
Mitochondria play a key role in iron metabolism in that they synthesize heme, assemble iron–
sulfur (Fe/S) proteins, and participate in cellular iron regulation. Here, we review the latter …
sulfur (Fe/S) proteins, and participate in cellular iron regulation. Here, we review the latter …
[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 …