The Iron−Sulfur Cluster of Pyruvate Formate-Lyase Activating Enzyme in Whole Cells: Cluster Interconversion and a Valence-Localized [4Fe-4S]2+ State

Biochemistry, Article ASAP

DOI: 10.1021/bi9010286

Publication Date (Web): August 27, 2009

Jian Yang‡, Sunil G. Naik§, Danilo O. Ortillo§, Ricardo Garca-Serres§, Meng Li‡, William E. Broderick, Boi Hanh Huynh*§ and Joan B. Broderick*

Department of Chemistry and Biochemistry, Montana State University, Bozeman, Montana 59718

^‡ Department of Chemistry, Michigan State University, East Lansing, Michigan 48824

^§ Department of Physics, Emory University, Atlanta, Georgia 30322

KEYWORDS: Pyruvate formate-lyase activating enzyme (PFL-AE),  glycyl radical, pyruvate formate-lyase (PFL),  [4Fe-4S] cluster, cluster interconversions,  [4Fe-4S]²⁺  , [2Fe-2S]²⁺ , Fe^III,  Fe^II,  redox state, 5′-deoxyadenosine, AMP, ADP, and methylthioadenosine, oxidative damage.

Recurrent Ventricular Tachycardia in Patient with Friedreich's Ataxia in the Absence of Clinical Myocardial Disease.

Pacing Clin Electrophysiol. 2009 Sep 10

Asaad N, El-Menyar A, Al Suwaidi J.

From the Department of Cardiology and Cardiovascular Surgery, Hamad General Hospital and Weill Cornell Medical College, Doha, Qatar.

Uptake of ferrous iron by cultured rat astrocytes

Journal of Neuroscience Research, Published Online: 10;Sep;2009


Ketki Tulpule ^{1 2}, Stephen R. Robinson ³, Glenda M. Bishop ³, Ralf Dringen ^{1 2 3 *1}Center for Biomolecular Interactions Bremen, University of Bremen, Bremen, Germany
²Center for Environmental Research and Sustainable Technology, Bremen, Germany
³School of Psychology, Psychiatry and Psychological Medicine, Monash University, Clayton, Victoria, Australia

TARGETING NUCLEIC ACIDS IN BACTERIA WITH SYNTHETIC LIGANDS

Abbas Nikravesh (Thesis for doctoral degree (Ph.D.))         
Stockholm 2008
From the Programme for Genomics and Bioinformatics
Department of Cell and Molecular Biology
Karolinska Institutet, Stockholm, Sweden



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3.4 PAPER IV
Friedreich’s ataxia (GAA)n triplex DNA structures are stabilized by Benzoquinoquinoxaline derivatives

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5 FUTURE PERSPECTIVES
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The experiments on (GAA)n repeat sequences described in this thesis were restricted to in vitro studies and probing of E. coli plasmid DNA. Nevertheless, the evidence that we provide for the existence of triplex structures within FA (GAA)n repeats suggests that destabilization of these structures could provide a possible therapeutic strategy for patients. Whereas BQQ stabilizes triplex structures, other DNA ligands may destabilize triplex structures. Such strategies may offer hope for developing small molecule DNA ligands to treat FA or other conditions that involve triplex DNA structures that arise from tri nucleotide repeat expansions.

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Structure-Specific Recognition of Friedreich's Ataxia (GAA)n Repeats by Benzoquinoquinoxaline Derivatives

ChemBioChem, Published Online: 10 Sep 2009

Helen Bergquist 1, Abbas Nikravesh, Dr. 2, Raquel Domingo Fernández 1, Veronica Larsson 1, Chi-Hung Nguyen, Dr. 3, Liam Good, Dr. 2 4, Rula Zain, Dr. 1 *


1Department of Molecular Biology and Functional Genomics, Stockholm University, Svante Arrhenius väg 20C, 10691 Stockholm (Sweden), Fax: (+46) 8-166488

2Department of Cell and Molecular Biology, Karolinska Institutet, Berzelius väg 35, 17177 Stockholm (Sweden)

3UMR176 CNRS-Institut Curie, Laboratoire de Pharmacochimie, Centre Universitaire, Bâtiment 110, 91405 Orsay (France)

4Department of Pathology and Infectious Diseases, Royal Veterinary College, University of London, Hawkshead Lane, N. Mymms AL9 7TA (UK)

Friedreich’s Ataxia Symposium 2009 - Children’s Hospital of Philadelphia

The symposium will take place on November 13 and 14.
Further information can be obtained from the Continuing Medical Education Department at the Children’s Hospital of Philadelphia at (215) 590 5263.

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

Proceedings of the National Academy of Science USA

Edited by Solomon H. Snyder, Johns Hopkins University School of Medicine, Baltimore, MD, and approved July 30, 2009 (received for review June 17, 2009)

Michael Li-Hsuan Huanga, Erika M. Beckera, Megan Whitnalla, Yohan Suryo Rahmantoa, Prem Ponkab,1 and Des R. Richardson.

Keywords: muscle creatine kinase (MCK), frataxin knockout mouse, frataxin deficiency, iron metabolism, neurologic and cardiologic degeneration, iron-sulfur cluster (ISC) synthesis, cysteine desulferase Nfs1, mitochondrial ferritin, 5-aminolevulinate dehydratase, coproporphyrinogen oxidase, hydroxymethylbilane synthase, uroporphyrinogen III synthase, ferrochelatase, Sec15l1, mitoferrin-2 (Mfrn2).

The roles of SbcCD and RNaseE in the transcription of GAA·TTC repeats in Escherichia coli

DNA Repair, article in press.

Xuefeng Pan^{a, b, ,} , Yunfeng Ding^{a, 1} and Lingfeng Shi^{a, 1}

^aSchool of Life Science, Beijing Institute of Technology, Beijing 100081, China

^bInstitute of Cell and Molecular Biology, Edinburgh University, Edinburgh EH9 3JR, Scotland, UK

Received 17 February 2009;

revised 24 July 2009;

accepted 9 August 2009.

Available online 4 September 2009.

Keyword: Frataxin, GAA·TTC repeats , FXN gene, Frederich's ataxia, α-Complementation SbcCD; RNaseE .

The dorsal root ganglion in Friedreich's ataxia.

Acta Neuropathol. 2009 Aug 30. [Epub ahead of print]

Koeppen AH, Morral JA, Davis AN, Qian J, Petrocine SV, Knutson MD, Gibson WM, Cusack MJ, Li D.
Research Service (151), Veterans Affairs Medical Center, 113 Holland Ave, Albany, NY, 12208, USA, arnulf.koeppen@med.va.gov.

Keywords: Atrophy of dorsal root ganglia (DRG), thinning of dorsal roots (DR), Friedreich's ataxia (FRDA), selective vulnerability of larger neurons in DRG, selective vulnerability of thicker myelinated DR axons, ventral roots (VR), Schwann cells, laminin, ferritin, mitochondrial ferritin, ferroportin.

AAV9-mediated erythropoietin gene delivery into the brain protects nigral dopaminergic neurons in a rat model of Parkinson's disease

Gene Therapy, advance online publication 3 September 2009; doi: 10.1038/gt.2009.113

Y-Q Xue1,7, B-F Ma1,7, L-R Zhao1,2,3, J B Tatom4, B Li2, L-X Jiang5, R L Klein3,4 and W-M Duan1,3,6
1Department of Cellular Biology and Anatomy, Louisiana State University Health Sciences Center, Shreveport, LA, USA
2Department of Neurology, Louisiana State University Health Sciences Center, Shreveport, LA, USA
3Gene Therapy Program, Louisiana State University Health Sciences Center, Shreveport, LA, USA
4Department of Pharmacology, Toxicology and Neuroscience, Louisiana State University Health Sciences Center, Shreveport, LA, USA
5Vector Gene Technology Company LTD, Beijing, PR China
6Department of Anatomy and Neurobiology, Capital Medical University, Beijing, PR China
Correspondence: Dr W-M Duan, Department of Cellular Biology & Anatomy, LSU Health Sciences Center in Shreveport, 1501 Kings Highway, Shreveport, LA 71130, USA. E-mail: wduan@lsuhsc.edu
7These authors contributed equally to this work.
Received 17 March 2009; Revised 3 August 2009; Accepted 5 August 2009; Published online 3 September 2009.