CoA‐dependent activation of mitochondrial acyl carrier protein links four neurodegenerative diseases

Safety and efficacy of deferiprone for pantothenate kinase-associated neurodegeneration: a randomised, double-blind, controlled trial and an open-label extension study

(2019) Thomas Klopstock et al.   LANCET NEUROLOGY

Protein lipoylation: an evolutionarily conserved metabolic regulator of health and disease

(2018) Elizabeth A Rowland et al.   CURRENT OPINION IN CHEMICAL BIOLOGY

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

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

Lipoic acid metabolism and mitochondrial redox regulation

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

Forces shaping the Drosophila wing

(2017) M.C. Diaz de la Loza et al.   MECHANISMS OF DEVELOPMENT

Structure of human Fe–S assembly subcomplex reveals unexpected cysteine desulfurase architecture and acyl-ACP–ISD11 interactions

(2017) Seth A. Cory et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Coenzyme A corrects pathological defects in human neurons of PANK2‐associated neurodegeneration

(2016) Daniel I Orellana et al.   EMBO Molecular Medicine

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

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

A Drosophila RNAi library modulates Hippo pathway-dependent tissue growth

(2016) Joseph H.A. Vissers et al.   Nature Communications

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

(2016) Jonathan G Van Vranken et al.   eLife

Mass spectral determination of phosphopantetheinylation specificity for carrier proteins in Mycobacterium tuberculosis

(2016) James Jung et al.   FEBS Open Bio

Extracellular 4′-phosphopantetheine is a source for intracellular coenzyme A synthesis

(2015) Balaji Srinivasan et al.   Nature Chemical Biology

Mitochondrial iron and energetic dysfunction distinguish fibroblasts and induced neurons from pantothenate kinase-associated neurodegeneration patients

(2015) Paolo Santambrogio et al.   NEUROBIOLOGY OF DISEASE

Sirtuin 4 Is a Lipoamidase Regulating Pyruvate Dehydrogenase Complex Activity

(2014) Rommel A. Mathias et al.   CELL

The Pyruvate Dehydrogenase Complexes: Structure-based Function and Regulation

(2014) Mulchand S. Patel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Architecture of mammalian respiratory complex I

(2014) Kutti R. Vinothkumar et al.   NATURE

A Drosophila RNAi collection is subject to dominant phenotypic effects

(2014) Edward W Green et al.   NATURE METHODS

Exome Sequence Reveals Mutations in CoA Synthase as a Cause of Neurodegeneration with Brain Iron Accumulation

(2013) Sabrina Dusi et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Pantethine treatment is effective in recovering the disease phenotype induced by ketogenic diet in a pantothenate kinase-associated neurodegeneration mouse model

(2013) Dario Brunetti et al.   BRAIN

Long-term safety of dichloroacetate in congenital lactic acidosis

(2013) Monica Abdelmalak et al.   MOLECULAR GENETICS AND METABOLISM

The phosphopantetheinyl transferases: catalysis of a post-translational modification crucial for life

(2013) Joris Beld et al.   NATURAL PRODUCT REPORTS

A genome-wide transgenic resource for conditional expression of Drosophila microRNAs

(2012) F. Bejarano et al.   DEVELOPMENT

Neuroimaging Features of Neurodegeneration with Brain Iron Accumulation

(2011) M.C. Kruer et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

Impaired Coenzyme A metabolism affects histone and tubulin acetylation inDrosophilaand human cell models of pantothenate kinase associated neurodegeneration

(2011) Katarzyna Siudeja et al.   EMBO Molecular Medicine

Metabolic consequences of mitochondrial coenzyme A deficiency in patients with PANK2 mutations

(2011) Valerio Leoni et al.   MOLECULAR GENETICS AND METABOLISM

Mitochondrial fatty acid synthesis and respiration

(2010) J. Kalervo Hiltunen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Pantethine rescues a Drosophila model for pantothenate kinase-associated neurodegeneration

(2010) A. Rana et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Metabolic Modulation of Glioblastoma with Dichloroacetate

(2010) E. D. Michelakis et al.   Science Translational Medicine

Pyruvate dehydrogenase E2 deficiency: A potentially treatable cause of episodic dystonia

(2009) Catherine A. McWilliam et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

Pantothenate kinase-associated neurodegeneration: insights from a Drosophila model

(2009) Z. Wu et al.   HUMAN MOLECULAR GENETICS

Acyl Carrier Protein-specific 4′-Phosphopantetheinyl Transferase Activates 10-Formyltetrahydrofolate Dehydrogenase

(2009) Kyle C. Strickland et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Down-regulation of Mitochondrial Acyl Carrier Protein in Mammalian Cells Compromises Protein Lipoylation and Respiratory Complex I and Results in Cell Death

(2009) Dejiang Feng et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

De novo CoA biosynthesis is required to maintain DNA integrity during development of the Drosophila nervous system

(2008) Floris Bosveld et al.   HUMAN MOLECULAR GENETICS