Mechanism of activation of the human cysteine desulfurase complex by frataxin

Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis

(2019) Tslil Ast et al.   CELL

Mechanism of frataxin “bypass” in human iron-sulfur cluster biosynthesis with implications for Friedreich’s ataxia

(2019) Deepika Das et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Synthetic transcription elongation factors license transcription across repressive chromatin

(2017) Graham S. Erwin et al.   SCIENCE

Structure and functional dynamics of the mitochondrial Fe/S cluster synthesis complex

(2017) Michal T. Boniecki et al.   Nature Communications

Labile Low-Molecular-Mass Metal Complexes in Mitochondria: Trials and Tribulations of a Burgeoning Field

(2016) Paul A. Lindahl et al.   BIOCHEMISTRY

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

(2016) Jonathan G Van Vranken et al.   eLife

Frataxin Accelerates [2Fe-2S] Cluster Formation on the Human Fe–S Assembly Complex

(2015) Nicholas G. Fox et al.   BIOCHEMISTRY

Interaction of frataxin, an iron binding protein, with IscU of Fe–S clusters biogenesis pathway and its upregulation in AmpB resistant Leishmania donovani

(2015) Amir Zaidi et al.   BIOCHIMIE

Mammalian frataxin directly enhances sulfur transfer of NFS1 persulfide to both ISCU and free thiols

(2015) Aubérie Parent et al.   Nature Communications

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

(2014) Jennifer Bridwell-Rabb et al.   BIOCHEMISTRY

Understanding Hydrogen Sulfide Storage: Probing Conditions for Sulfide Release from Hydrodisulfides

(2014) T. Spencer Bailey et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

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

(2014) Tracey A. Rouault   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Functional reconstitution of mitochondrial Fe/S cluster synthesis on Isu1 reveals the involvement of ferredoxin

(2014) Holger Webert et al.   Nature Communications

The effect of the adaptor protein Isd11 on the quaternary structure of the eukaryotic cysteine desulphurase Nfs1

(2013) Kerem Teralı et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Frataxin Directly Stimulates Mitochondrial Cysteine Desulfurase by Exposing Substrate-binding Sites, and a Mutant Fe-S Cluster Scaffold Protein with Frataxin-bypassing Ability Acts Similarly

(2013) Alok Pandey et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Role of Mitochondria in Cellular Iron-Sulfur Protein Biogenesis: Mechanisms, Connected Processes, and Diseases

(2013) O. Stehling et al.   Cold Spring Harbor Perspectives in Biology

Effector Role Reversal during Evolution: The Case of Frataxin in Fe–S Cluster Biosynthesis

(2012) Jennifer Bridwell-Rabb et al.   BIOCHEMISTRY

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

Missense Mutations Linked to Friedreich Ataxia Have Different but Synergistic Effects on Mitochondrial Frataxin Isoforms

(2012) Hongqiao Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mammalian Frataxin Controls Sulfur Production and Iron Entry during de Novo Fe4S4 Cluster Assembly

(2012) Florent Colin et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Structural and Functional Studies of the Mitochondrial Cysteine Desulfurase from Arabidopsis thaliana

(2012) Valeria R. Turowski et al.   Molecular Plant

Friedreich’s Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron–Sulfur Cluster Assembly Complex

(2011) Chi-Lin Tsai et al.   BIOCHEMISTRY

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

Friedreich's ataxia: Past, present and future

(2011) Daniele Marmolino   BRAIN RESEARCH REVIEWS

A Combined Nucleic Acid and Protein Analysis in Friedreich Ataxia: Implications for Diagnosis, Pathogenesis and Clinical Trial Design

(2011) Francesco Saccà et al.   PLoS One

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

Frataxin, a molecule of mystery: trading stability for function in its iron-binding site: Figure 1

(2010) Darius J. R. Lane et al.   BIOCHEMICAL JOURNAL

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

(2010) Chi-Lin Tsai et al.   BIOCHEMISTRY

Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches

(2010) S. Schmucker et al.   HUMAN MOLECULAR GENETICS

Normal and Friedreich Ataxia Cells Express Different Isoforms of Frataxin with Complementary Roles in Iron-Sulfur Cluster Assembly

(2010) Oleksandr Gakh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A rapid, noninvasive immunoassay for frataxin: Utility in assessment of Friedreich ataxia

(2010) Eric C. Deutsch et al.   MOLECULAR GENETICS AND METABOLISM

Building Fe–S proteins: bacterial strategies

(2010) Béatrice Py et al.   NATURE REVIEWS MICROBIOLOGY

A high throughput electrochemiluminescence assay for the quantification of frataxin protein levels

(2009) Hannes Steinkellner et al.   ANALYTICA CHIMICA ACTA

Kinetic Analysis of Cysteine Desulfurase CD0387 fromSynechocystissp. PCC 6803: Formation of the Persulfide Intermediate

(2009) Elham Behshad et al.   BIOCHEMISTRY

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

Oligomeric Yeast Frataxin Drives Assembly of Core Machinery for Mitochondrial Iron-Sulfur Cluster Synthesis

(2009) Hongqiao Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Function and biogenesis of iron–sulphur proteins

(2009) Roland Lill   NATURE

Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS

(2009) Salvatore Adinolfi et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

DrosophilaFrataxin: An Iron Chaperone during Cellular Fe−S Cluster Bioassembly†

(2008) Kalyan C. Kondapalli et al.   BIOCHEMISTRY

Structural Basis of the Iron Storage Function of Frataxin from Single-Particle Reconstruction of the Iron-Loaded Oligomer†

(2008) Ulrika Schagerlöf et al.   BIOCHEMISTRY

The in vivo mitochondrial two-step maturation of human frataxin

(2008) Stéphane Schmucker et al.   HUMAN MOLECULAR GENETICS

A Novel Role for Human Nfs1 in the Cytoplasm

(2008) Zvonimir Marelja et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mapping iron binding sites on human frataxin: implications for cluster assembly on the ISU Fe–S cluster scaffold protein

(2008) Jia Huang et al.   JOURNAL OF BIOLOGICAL INORGANIC CHEMISTRY

Lateral-flow immunoassay for the frataxin protein in Friedreich’s ataxia patients and carriers

(2008) John H. Willis et al.   MOLECULAR GENETICS AND METABOLISM