Role of Nfu1 and Bol3 in iron-sulfur cluster transfer to mitochondrial clients

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

(2015) Nicholas G. Fox et al.   BIOCHEMISTRY

Mutation of the iron-sulfur cluster assembly gene IBA57 causes fatal infantile leukodystrophy

(2015) François-Guillaume Debray et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Fe/S protein assembly gene IBA57 mutation causes hereditary spastic paraplegia

(2015) A. Lossos et al.   NEUROLOGY

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

(2015) Aubérie Parent et al.   Nature Communications

The structure of lipoyl synthase, a remarkable enzyme that performs the last step of an extraordinary biosynthetic pathway

(2014) John E. Cronan   BIOCHEMICAL JOURNAL

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

(2014) Jennifer Bridwell-Rabb et al.   BIOCHEMISTRY

The LYR Factors SDHAF1 and SDHAF3 Mediate Maturation of the Iron-Sulfur Subunit of Succinate Dehydrogenase

(2014) Un Na et al.   Cell Metabolism

Structural and Spectroscopic Insights into BolA-Glutaredoxin Complexes

(2014) Thomas Roret et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ISCA2mutation causes infantile neurodegenerative mitochondrial disorder

(2014) Zuhair N Al-Hassnan et al.   JOURNAL OF MEDICAL GENETICS

Formation of [4Fe-4S] Clusters in the Mitochondrial Iron–Sulfur Cluster Assembly Machinery

(2014) Diego Brancaccio et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Leukoencephalopathy with cysts and hyperglycinemia may result from NFU1 deficiency

(2014) Mathilde Nizon et al.   MITOCHONDRION

[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

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

(2014) Holger Webert et al.   Nature Communications

Arabidopsis thaliana Nfu2 Accommodates [2Fe-2S] or [4Fe-4S] Clusters and Is Competent for in Vitro Maturation of Chloroplast [2Fe-2S] and [4Fe-4S] Cluster-Containing Proteins

(2013) Huanyao Gao et al.   BIOCHEMISTRY

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

(2013) Peter R. Baker et al.   BRAIN

Physical and Functional Interactions of a Monothiol Glutaredoxin and an Iron Sulfur Cluster Carrier Protein with the Sulfur-donating RadicalS-Adenosyl-l-methionine Enzyme MiaB

(2013) Sylvain Boutigny et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

The mitochondrial Hsp70 chaperone Ssq1 facilitates Fe/S cluster transfer from Isu1 to Grx5 by complex formation

(2013) Marta A. Uzarska et al.   MOLECULAR BIOLOGY OF THE CELL

BOLA1 Is an Aerobic Protein That Prevents Mitochondrial Morphology Changes Induced by Glutathione Depletion

(2012) Peter Willems et al.   ANTIOXIDANTS & REDOX SIGNALING

Monothiol CGFS Glutaredoxins and BolA-like Proteins: [2Fe-2S] Binding Partners in Iron Homeostasis

(2012) Haoran Li et al.   BIOCHEMISTRY

Human Glutaredoxin 3 Forms [2Fe-2S]-Bridged Complexes with Human BolA2

(2012) Haoran Li 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

Flavinylation and Assembly of Succinate Dehydrogenase Are Dependent on the C-terminal Tail of the Flavoprotein Subunit

(2012) Hyung J. Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Protein expression profiles in patients carrying NFU1 mutations. Contribution to the pathophysiology of the disease

(2012) Xènia Ferrer-Cortès et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Interaction of J-Protein Co-Chaperone Jac1 with Fe–S Scaffold Isu Is Indispensable In Vivo and Conserved in Evolution

(2012) Szymon J. Ciesielski et al.   JOURNAL OF MOLECULAR BIOLOGY

Optimized Fast and Sensitive Acquisition Methods for Shotgun Proteomics on a Quadrupole Orbitrap Mass Spectrometer

(2012) Christian D. Kelstrup et al.   JOURNAL OF PROTEOME RESEARCH

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

The fungal α-aminoadipate pathway for lysine biosynthesis requires two enzymes of the aconitase family for the isomerization of homocitrate to homoisocitrate

(2012) Felicitas Fazius et al.   MOLECULAR MICROBIOLOGY

Molecular organization, biochemical function, cellular role and evolution of NfuA, an atypical Fe-S carrier

(2012) Béatrice Py et al.   MOLECULAR MICROBIOLOGY

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

Specialized Function of Yeast Isa1 and Isa2 Proteins in the Maturation of Mitochondrial [4Fe-4S] Proteins

(2011) Ulrich Mühlenhoff et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mass Spectrometry-based Proteomics Using Q Exactive, a High-performance Benchtop Quadrupole Orbitrap Mass Spectrometer

(2011) Annette Michalski et al.   MOLECULAR & CELLULAR PROTEOMICS

The LYR Protein Mzm1 Functions in the Insertion of the Rieske Fe/S Protein in Yeast Mitochondria

(2011) A. Atkinson et al.   MOLECULAR AND CELLULAR BIOLOGY

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

Monothiol glutaredoxin Grx5 interacts with Fe–S scaffold proteins Isa1 and Isa2 and supports Fe–S assembly and DNA integrity in mitochondria of fission yeast

(2010) Kyoung-Dong Kim et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

(2010) Chi-Lin Tsai et al.   BIOCHEMISTRY

Mitochondrial fatty acid synthesis and respiration

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

Iron−Sulfur Cluster Biosynthesis: Functional Characterization of the N- and C-Terminal Domains of Human NFU†

(2009) Yushi Liu et al.   BIOCHEMISTRY

Maturation of Iron-Sulfur Proteins in Eukaryotes: Mechanisms, Connected Processes, and Diseases

(2008) Roland Lill et al.   Annual Review of Biochemistry

NfuA, a New Factor Required for Maturing Fe/S Proteins inEscherichia coliunder Oxidative Stress and Iron Starvation Conditions

(2008) Sandra Angelini et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial Fatty Acid Synthesis Type II: More than Just Fatty Acids

(2008) J. Kalervo Hiltunen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Proposed Role for theAzotobacter vinelandiiNfuA Protein as an Intermediate Iron-Sulfur Cluster Carrier

(2008) Sibali Bandyopadhyay 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