Genetic dissection of the mitochondrial lipoylation pathway in yeast

Progress in the Enzymology of the Mitochondrial Diseases of Lipoic Acid Requiring Enzymes

(2020) John E. Cronan   Frontiers in Genetics

Mitochondrial Fatty Acids and Neurodegenerative Disorders

(2020) Alexander J. Kastaniotis et al.   NEUROSCIENTIST

Non-canonical translation initiation in yeast generates a cryptic pool of mitochondrial proteins

(2019) Geoffray Monteuuis et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial acyl carrier protein (ACP) at the interface of metabolic state sensing and mitochondrial function

(2019) Ali J. Masud et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Novel mutations in malonyl-CoA-acyl carrier protein transacylase provoke autosomal recessive optic neuropathy

(2019) Huiping Li et al.   HUMAN MOLECULAR GENETICS

Development and retention of a primordial moonlighting pathway of protein modification in the absence of selection presents a puzzle

(2018) Xinyun Cao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ACP Acylation Is an Acetyl-CoA-Dependent Modification Required for Electron Transport Chain Assembly

(2018) Jonathan G. Van Vranken et al.   MOLECULAR CELL

Genetic modifications of Mecr reveal a role for mitochondrial 2-enoyl-CoA/ACP reductase in placental development in mice

(2017) Remya R. Nair et al.   HUMAN MOLECULAR GENETICS

Lipoic acid metabolism and mitochondrial redox regulation

(2017) Ashley Solmonson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cox16 protein is physically associated with Cox1p assembly intermediates and with cytochrome oxidase

(2017) Chen-Hsien Su et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2017) Erin L. McCarthy et al.   SCIENCE

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

The mitochondrial acyl carrier protein (ACP) coordinates mitochondrial fatty acid synthesis with iron sulfur cluster biogenesis

(2016) Jonathan G Van Vranken et al.   eLife

Clinical, biochemical, and genetic spectrum of seven patients with NFU1 deficiency

(2015) Uwe Ahting et al.   Frontiers in Genetics

4-Methylene-2-octyl-5-oxotetrahydrofuran-3-carboxylic Acid (C75), an Inhibitor of Fatty-acid Synthase, Suppresses the Mitochondrial Fatty Acid Synthesis Pathway and Impairs Mitochondrial Function

(2014) Cong Chen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lipoic acid biosynthesis defects

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

Expression and Evolution of the Non-Canonically Translated Yeast Mitochondrial Acetyl-CoA Carboxylase Hfa1p

(2014) Fumi Suomi et al.   PLoS One

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

(2013) Peter R. Baker et al.   BRAIN

Mutations in the lipoyltransferase LIPT1 gene cause a fatal disease associated with a specific lipoylation defect of the 2-ketoacid dehydrogenase complexes

(2013) Frederic Tort et al.   HUMAN MOLECULAR GENETICS

Defects in mitochondrial fatty acid synthesis result in failure of multiple aspects of mitochondrial biogenesis inSaccharomyces cerevisiae

(2013) V. A. Samuli Kursu et al.   MOLECULAR MICROBIOLOGY

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

Mutations in Iron-Sulfur Cluster Scaffold Genes NFU1 and BOLA3 Cause a Fatal Deficiency of Multiple Respiratory Chain and 2-Oxoacid Dehydrogenase Enzymes

(2011) Jessie M. Cameron et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Lipoic Acid Synthetase Deficiency Causes Neonatal-Onset Epilepsy, Defective Mitochondrial Energy Metabolism, and Glycine Elevation

(2011) Johannes A. Mayr et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A Fatal Mitochondrial Disease Is Associated with Defective NFU1 Function in the Maturation of a Subset of Mitochondrial Fe-S Proteins

(2011) Aleix Navarro-Sastre et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A novel two-gene requirement for the octanoyltransfer reaction of Bacillus subtilis lipoic acid biosynthesis

(2011) Natalia Martin et al.   MOLECULAR MICROBIOLOGY

Scavenging of Cytosolic Octanoic Acid by Mutant LplA Lipoate Ligases Allows Growth of Escherichia coli Strains Lacking the LipB Octanoyltransferase of Lipoic Acid Synthesis

(2009) F. A. M. Hermes et al.   JOURNAL OF BACTERIOLOGY

Lipoic Acid Synthesis and Attachment in Yeast Mitochondria

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

Intersection of RNA Processing and the Type II Fatty Acid Synthesis Pathway in Yeast Mitochondria

(2008) M. S. Schonauer et al.   MOLECULAR AND CELLULAR BIOLOGY