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

A Mammalian Siderophore Synthesized by an Enzyme with a Bacterial Homolog Involved in Enterobactin Production

(2010) Laxminarayana R. Devireddy et al.   CELL

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

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

Frataxin deficiency induces Schwann cell inflammation and death

(2009) Chunye Lu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARγ pathway as a therapeutic target in Friedreich’s ataxia

(2009) Giovanni Coppola 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

Friedreich's ataxia: Oxidative stress and cytoskeletal abnormalities

(2009) Marco Sparaco et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

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

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

Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia

(2009) Vincent Paupe et al.   PLoS One

Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant

(2009) M. L.-H. Huang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

DNA triplexes and Friedreich ataxia

(2008) Robert D. Wells   FASEB JOURNAL

The in vivo mitochondrial two-step maturation of human frataxin

(2008) Stéphane Schmucker et al.   HUMAN MOLECULAR GENETICS

Binding of Yeast Frataxin to the Scaffold for Fe-S Cluster Biogenesis, Isu

(2008) Tao Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Novel Role for Human Nfs1 in the Cytoplasm

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

The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation

(2008) M. Whitnall et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial localization of human frataxin is necessary but processing is not for rescuing frataxin deficiency in Trypanosoma brucei

(2008) S. Long et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Iron–sulfur cluster biogenesis and human disease

(2008) Tracey A. Rouault et al.   TRENDS IN GENETICS