Lipoic acid metabolism and mitochondrial redox regulation

Human dihydrolipoamide dehydrogenase (E3) deficiency: Novel insights into the structural basis and molecular pathomechanism

(2018) Attila Ambrus et al.   NEUROCHEMISTRY INTERNATIONAL

Protein S-glutathionylation alters superoxide/hydrogen peroxide emission from pyruvate dehydrogenase complex

(2017) Marisa O’Brien et al.   FREE RADICAL BIOLOGY AND MEDICINE

Destruction and reformation of an iron-sulfur cluster during catalysis by lipoyl synthase

(2017) Erin L. McCarthy et al.   SCIENCE

Sirtuin Lipoamidase Activity Is Conserved in Bacteria as a Regulator of Metabolic Enzyme Complexes

(2017) Elizabeth A. Rowland et al.   mBio

MECR Mutations Cause Childhood-Onset Dystonia and Optic Atrophy, a Mitochondrial Fatty Acid Synthesis Disorder

(2016) Gali Heimer et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial Protein Lipoylation and the 2-Oxoglutarate Dehydrogenase Complex Controls HIF1α Stability in Aerobic Conditions

(2016) Stephen P. Burr et al.   Cell Metabolism

Production of superoxide/hydrogen peroxide by the mitochondrial 2-oxoadipate dehydrogenase complex

(2016) Renata L.S. Goncalves et al.   FREE RADICAL BIOLOGY AND MEDICINE

Assembly of Lipoic Acid on Its Cognate Enzymes: an Extraordinary and Essential Biosynthetic Pathway

(2016) John E. Cronan   MICROBIOLOGY AND MOLECULAR BIOLOGY REVIEWS

Induction of mitochondrial reactive oxygen species production by GSH mediated S-glutathionylation of 2-oxoglutarate dehydrogenase

(2016) Ryan J. Mailloux et al.   Redox Biology

Hypoxia Induces Production of L-2-Hydroxyglutarate

(2015) Andrew M. Intlekofer et al.   Cell Metabolism

Sirtuin 4 Is a Lipoamidase Regulating Pyruvate Dehydrogenase Complex Activity

(2014) Rommel A. Mathias et al.   CELL

Lysine Glutarylation Is a Protein Posttranslational Modification Regulated by SIRT5

(2014) Minjia Tan et al.   Cell Metabolism

The 2-Oxoacid Dehydrogenase Complexes in Mitochondria Can Produce Superoxide/Hydrogen Peroxide at Much Higher Rates Than Complex I

(2014) Casey L. Quinlan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lipoic acid biosynthesis defects

(2014) Johannes A. Mayr et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Lipoate-Protein Ligase and Octanoyltransferase Are Essential for Protein Lipoylation in Mitochondria of Arabidopsis

(2014) R. Ewald et al.   PLANT PHYSIOLOGY

Variant non ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5

(2013) Peter R. Baker et al.   BRAIN

DHTKD1 is essential for mitochondrial biogenesis and function maintenance

(2013) Wangyang Xu et al.   FEBS LETTERS

Glutathionylation of α-ketoglutarate dehydrogenase: The chemical nature and relative susceptibility of the cofactor lipoic acid to modification

(2013) Aaron L. McLain et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial glutathione depletion reveals a novel role for the pyruvate dehydrogenase complex as a key H2O2-emitting source under conditions of nutrient overload

(2013) Kelsey H. Fisher-Wellman et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase

(2013) Yohan Soreze et al.   Orphanet Journal of Rare Diseases

The role of theSaccharomyces cerevisiaelipoate protein ligase homologue, Lip3, in lipoic acid synthesis

(2013) Fatemah A. Hermes et al.   YEAST

DHTKD1 Mutations Cause 2-Aminoadipic and 2-Oxoadipic Aciduria

(2012) Katharina Danhauser et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Compromised Mitochondrial Fatty Acid Synthesis in Transgenic Mice Results in Defective Protein Lipoylation and Energy Disequilibrium

(2012) Stuart Smith et al.   PLoS One

Glutaredoxin 2 prevents H2O2-induced cell apoptosis by protecting complex I activity in the mitochondria

(2010) Hongli Wu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Inhibition of the α-ketoglutarate dehydrogenase-mediated reactive oxygen species generation by lipoic acid

(2010) Attila Ambrus et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Lipoic Acid Synthesis and Attachment in Yeast Mitochondria

(2009) Melissa S. Schonauer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Reversible Inhibition of α-Ketoglutarate Dehydrogenase by Hydrogen Peroxide:  Glutathionylation and Protection of Lipoic Acid†

(2007) Milana A. B. Applegate et al.   BIOCHEMISTRY