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

Frataxin-bypassing Isu1: characterization of the bypass activity in cells and mitochondria

(2014) Heeyong Yoon et al.   BIOCHEMICAL JOURNAL

Electron-Transfer Pathways in the Heme and Quinone-Binding Domain of Complex II (Succinate Dehydrogenase)

(2014) Robert F. Anderson et al.   BIOCHEMISTRY

Human Frataxin Activates Fe–S Cluster Biosynthesis by Facilitating Sulfur Transfer Chemistry

(2014) Jennifer Bridwell-Rabb et al.   BIOCHEMISTRY

Evidence for a Catalytically and Kinetically Competent Enzyme–Substrate Cross-Linked Intermediate in Catalysis by Lipoyl Synthase

(2014) Nicholas D. Lanz et al.   BIOCHEMISTRY

Mitochondrial iron–sulfur protein biogenesis and human disease

(2014) Oliver Stehling et al.   BIOCHIMIE

Cochaperone Binding to LYR Motifs Confers Specificity of Iron Sulfur Cluster Delivery

(2014) Nunziata Maio et al.   Cell Metabolism

A structural model for glutathione-complexed iron–sulfur cluster as a substrate for ABCB7-type transporters

(2014) Wenbin Qi et al.   CHEMICAL COMMUNICATIONS

A journey into the active center of nitrogenase

(2014) Yilin Hu et al.   JOURNAL OF BIOLOGICAL INORGANIC CHEMISTRY

DNA-Mediated Signaling by Proteins with 4Fe–4S Clusters Is Necessary for Genomic Integrity

(2014) Michael A. Grodick et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Role of IscX in Iron–Sulfur Cluster Biogenesis in Escherichia coli

(2014) Jin Hae Kim et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich's ataxia

(2014) Morgane Perdomini et al.   NATURE MEDICINE

[2Fe-2S] cluster transfer in iron-sulfur protein biogenesis

(2014) L. Banci et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Pathophysiogical and therapeutic progress in Friedreich ataxia

(2014) H. Puccio et al.   REVUE NEUROLOGIQUE

Crystal Structures of Nucleotide-Free and Glutathione-Bound Mitochondrial ABC Transporter Atm1

(2014) V. Srinivasan et al.   SCIENCE

Structural Basis for Heavy Metal Detoxification by an Atm1-Type ABC Exporter

(2014) J. Y. Lee et al.   SCIENCE

Mitochondrial Complex I

(2013) Judy Hirst   Annual Review of Biochemistry

The superfamily of mitochondrial Complex1_LYR motif-containing (LYRM) proteins

(2013) Heike Angerer   BIOCHEMICAL SOCIETY TRANSACTIONS

Reprint of: Iron/sulfur proteins biogenesis in prokaryotes: Formation, regulation and diversity

(2013) Béatrice Roche et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

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

(2013) Peter R. Baker et al.   BRAIN

Human CIA2A-FAM96A and CIA2B-FAM96B Integrate Iron Homeostasis and Maturation of Different Subsets of Cytosolic-Nuclear Iron-Sulfur Proteins

(2013) Oliver Stehling et al.   Cell Metabolism

Deleterious mutation in FDX1L gene is associated with a novel mitochondrial muscle myopathy

(2013) Ronen Spiegel et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Mortalin is a prognostic factor of gastric cancer with normal p53 function

(2013) Koji Ando et al.   Gastric Cancer

Mutations in LYRM4, encoding iron–sulfur cluster biogenesis factor ISD11, cause deficiency of multiple respiratory chain complexes

(2013) Sze Chern Lim et al.   HUMAN MOLECULAR GENETICS

Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy

(2013) Nikhita Ajit Bolar et al.   HUMAN MOLECULAR GENETICS

Binding of the Chaperone Jac1 Protein and Cysteine Desulfurase Nfs1 to the Iron-Sulfur Cluster Scaffold Isu Protein Is Mutually Exclusive

(2013) Julia Majewska et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human Mitochondrial Chaperone (mtHSP70) and Cysteine Desulfurase (NFS1) Bind Preferentially to the Disordered Conformation, Whereas Co-chaperone (HSC20) Binds to the Structured Conformation of the Iron-Sulfur Cluster Scaffold Protein (ISCU)

(2013) Kai Cai et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Frataxin: a protein in search for a function

(2013) Annalisa Pastore et al.   JOURNAL OF NEUROCHEMISTRY

Clinical features of Friedreich's ataxia: classical and atypical phenotypes

(2013) Michael H. Parkinson et al.   JOURNAL OF NEUROCHEMISTRY

Monothiol Glutaredoxins Can Bind Linear [Fe3S4]+ and [Fe4S4]2+ Clusters in Addition to [Fe2S2]2+ Clusters: Spectroscopic Characterization and Functional Implications

(2013) Bo Zhang et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

[2Fe-2S]-Ferredoxin Binds Directly to Cysteine Desulfurase and Supplies an Electron for Iron–Sulfur Cluster Assembly but Is Displaced by the Scaffold Protein or Bacterial Frataxin

(2013) Jin Hae Kim et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

The Evolutionarily Conserved Iron-Sulfur Protein INDH Is Required for Complex I Assembly and Mitochondrial Translation in Arabidopsis

(2013) M. M. Wydro et al.   PLANT CELL

The L-Cysteine Desulfurase NFS1 Is Localized in the Cytosol where it Provides the Sulfur for Molybdenum Cofactor Biosynthesis in Humans

(2013) Zvonimir Marelja et al.   PLoS One

Maturation of cytosolic and nuclear iron–sulfur proteins

(2013) Daili J.A. Netz et al.   TRENDS IN CELL BIOLOGY

(IscS-IscU)2Complex Structures Provide Insights into Fe2S2Biogenesis and Transfer

(2012) Elodie N. Marinoni et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

Persulfide formation on mitochondrial cysteine desulfurase: enzyme activation by a eukaryote-specific interacting protein and Fe–S cluster synthesis

(2012) Alok Pandey et al.   BIOCHEMICAL JOURNAL

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

(2012) Roland Lill et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Radical SAM enzymes and radical enzymology

(2012) Squire J. Booker   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease

(2012) T. A. Rouault   Disease Models & Mechanisms

X-linked sideroblastic anemia and ataxia: A new family with identification of a fourth ABCB7 gene mutation

(2012) Marc D’Hooghe et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

Tissue Specificity of a Human Mitochondrial Disease

(2012) Daniel R. Crooks et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Bridging [4Fe-4S] Cluster and Nucleotide Binding Are Essential for Function of the Cfd1-Nbp35 Complex as a Scaffold in Iron-Sulfur Protein Maturation

(2012) Daili J. A. Netz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Homozygous missense mutation in BOLA3 causes multiple mitochondrial dysfunctions syndrome in two siblings

(2012) Tobias B. Haack et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Glutathione Complexed Fe–S Centers

(2012) Wenbin Qi et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation

(2012) Alex D. Sheftel et al.   MOLECULAR BIOLOGY OF THE CELL

MMS19 Assembles Iron-Sulfur Proteins Required for DNA Metabolism and Genomic Integrity

(2012) O. Stehling et al.   SCIENCE

MMS19 Links Cytoplasmic Iron-Sulfur Cluster Assembly to DNA Metabolism

(2012) K. Gari et al.   SCIENCE

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

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

Structure–Function Analysis of Friedreich’s Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe–S Assembly Complex

(2011) Jennifer Bridwell-Rabb et al.   BIOCHEMISTRY

Biophysical probes of iron metabolism in cells and organelles

(2011) Paul A Lindahl et al.   CURRENT OPINION IN CHEMICAL BIOLOGY

Mouse Knock-out of IOP1 Protein Reveals Its Essential Role in Mammalian Cytosolic Iron-Sulfur Protein Biogenesis

(2011) Daisheng Song et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Leucine Biosynthesis Regulates Cytoplasmic Iron-Sulfur Enzyme Biogenesis in an Atm1p-independent Manner

(2011) Tibor Bedekovics et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mammalian Frataxin: An Essential Function for Cellular Viability through an Interaction with a Preformed ISCU/NFS1/ISD11 Iron-Sulfur Assembly Complex

(2011) Stéphane Schmucker et al.   PLoS One

Human Frataxin Is an Allosteric Switch That Activates the Fe−S Cluster Biosynthetic Complex

(2010) Chi-Lin Tsai et al.   BIOCHEMISTRY

Characterization of the human HSC20, an unusual DnaJ type III protein, involved in iron–sulfur cluster biogenesis

(2010) Helge Uhrigshardt et al.   HUMAN MOLECULAR GENETICS

Tah18 transfers electrons to Dre2 in cytosolic iron-sulfur protein biogenesis

(2010) Daili J A Netz et al.   Nature Chemical Biology

The HSP70 chaperone machinery: J proteins as drivers of functional specificity

(2010) Harm H. Kampinga et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Structural bases for the interaction of frataxin with the central components of iron–sulphur cluster assembly

(2010) Filippo Prischi et al.   Nature Communications

Structural Basis for Fe–S Cluster Assembly and tRNA Thiolation Mediated by IscS Protein–Protein Interactions

(2010) Rong Shi et al.   PLOS BIOLOGY

Clinical manifestation and a new ISCU mutation in iron–sulphur cluster deficiency myopathy

(2009) Gittan Kollberg et al.   BRAIN

Human ISD11 is essential for both iron-sulfur cluster assembly and maintenance of normal cellular iron homeostasis

(2009) Y. Shi et al.   HUMAN MOLECULAR GENETICS

Human Ind1, an Iron-Sulfur Cluster Assembly Factor for Respiratory Complex I

(2009) A. D. Sheftel et al.   MOLECULAR AND CELLULAR BIOLOGY

SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy

(2009) Daniele Ghezzi et al.   NATURE GENETICS

Splice Mutation in the Iron-Sulfur Cluster Scaffold Protein ISCU Causes Myopathy with Exercise Intolerance

(2008) Fanny Mochel et al.   AMERICAN JOURNAL OF HUMAN GENETICS

The iron–sulphur protein Ind1 is required for effective complex I assembly

(2008) Katrine Bych et al.   EMBO JOURNAL

Myopathy with lactic acidosis is linked to chromosome 12q23.3-24.11 and caused by an intron mutation in the ISCU gene resulting in a splicing defect

(2008) A. Olsson et al.   HUMAN MOLECULAR GENETICS

Human Nbp35 Is Essential for both Cytosolic Iron-Sulfur Protein Assembly and Iron Homeostasis

(2008) O. Stehling et al.   MOLECULAR AND CELLULAR BIOLOGY