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) …

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 …

[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 …

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 …

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 …

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 …

[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 …

Mitochondrial respiratory chain complexes

JS Sousa, E D'Imprima, J Vonck - Membrane protein complexes: structure …, 2018 - Springer
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 …

[HTML][HTML] The role of mitochondria in cellular iron–sulfur protein biogenesis and iron metabolism

R Lill, B Hoffmann, S Molik, AJ Pierik… - … et Biophysica Acta (BBA …, 2012 - Elsevier
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 …

[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 …