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 …

Mitochondria and Iron: current questions

BT Paul, DH Manz, FM Torti, SV Torti - Expert review of hematology, 2017 - Taylor & Francis
Introduction: Mitochondria are cellular organelles that perform numerous bioenergetic,
biosynthetic, and regulatory functions and play a central role in iron metabolism …

Mitochondrial iron–sulfur protein biogenesis and human disease

O Stehling, C Wilbrecht, R Lill - Biochimie, 2014 - Elsevier
Work during the past 14 years has shown that mitochondria are the primary site for the
biosynthesis of iron–sulfur (Fe/S) clusters. In fact, it is this process that renders mitochondria …

The role of mitochondria in cellular iron–sulfur protein biogenesis: mechanisms, connected processes, and diseases

O Stehling, R Lill - Cold Spring Harbor perspectives in …, 2013 - cshperspectives.cshlp.org
Iron–sulfur (Fe/S) clusters belong to the most ancient protein cofactors in life, and fulfill
functions in electron transport, enzyme catalysis, homeostatic regulation, and sulfur …

Lipoic acid biosynthesis defects

JA Mayr, RG Feichtinger, F Tort, A Ribes… - Journal of inherited …, 2014 - Springer
Lipoate is a covalently bound cofactor essential for five redox reactions in humans: in four 2-
oxoacid dehydrogenases and the glycine cleavage system (GCS). Two enzymes are from …

[HTML][HTML] Iron–sulfur cluster biogenesis in mammalian cells: new insights into the molecular mechanisms of cluster delivery

N Maio, TA Rouault - Biochimica et Biophysica Acta (BBA)-Molecular Cell …, 2015 - Elsevier
Abstract Iron–sulfur (Fe–S) clusters are ancient, ubiquitous cofactors composed of iron and
inorganic sulfur. The combination of the chemical reactivity of iron and sulfur, together with …

Mammalian iron–sulphur proteins: novel insights into biogenesis and function

TA Rouault - Nature reviews Molecular cell biology, 2015 - nature.com
Abstract Iron–sulphur (Fe–S) clusters are inorganic cofactors that are found in nearly all
species and are composed of various combinations of iron and sulphur atoms. Fe–S clusters …

Mitochondrial fatty acid synthesis, fatty acids and mitochondrial physiology

AJ Kastaniotis, KJ Autio, JM Kerätär… - … et Biophysica Acta (BBA …, 2017 - Elsevier
Mitochondria and fatty acids are tightly connected to a multiplicity of cellular processes that
go far beyond mitochondrial fatty acid metabolism. In line with this view, there is hardly any …

Mitochondrial [4Fe-4S] protein assembly involves reductive [2Fe-2S] cluster fusion on ISCA1–ISCA2 by electron flow from ferredoxin FDX2

BD Weiler, MC Brück, I Kothe, E Bill… - Proceedings of the …, 2020 - National Acad Sciences
The essential process of iron-sulfur (Fe/S) cluster assembly (ISC) in mitochondria occurs in
three major phases. First,[2Fe-2S] clusters are synthesized on the scaffold protein ISCU2; …

Cochaperone binding to LYR motifs confers specificity of iron sulfur cluster delivery

N Maio, A Singh, H Uhrigshardt, N Saxena, WH Tong… - Cell metabolism, 2014 - cell.com
Iron sulfur (Fe-S) clusters, preassembled on the ISCU scaffold, are transferred to target
proteins or to intermediate scaffolds by a dedicated chaperone-cochaperone system …