Impaired inhibition of prepotent motor tendencies in Friedreich ataxia demonstrated by the Simon interference task.

Brain Cogn. 2011 Feb 25

Corben LA, Akhlaghi H, Georgiou-Karistianis N, Bradshaw JL, Egan GF, Storey E, Churchyard AJ, Delatycki MB.

Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, Victoria, Australia; Experimental Neuropsychology Research Unit, School of Psychology and Psychiatry, Monash University, Clayton 3800, Victoria, Australia.

KEYWORDS: Friedreich ataxia (FRDA), impairment in motor planning, cerebral cortex and/or cerebello-cortical projections, Simon interference task.

Human Basal Ganglia and the Dynamic Control of Force during On-Line Corrections

The Journal of Neuroscience, February 2, 2011, 31(5):1600-1605; doi:10.1523/JNEUROSCI.3301-10.2011

Scott T. Grafton1 and Eugene Tunik2

1Sage Center for the Study of Mind, Department of Psychology, University of California, Santa Barbara, California 93106, and 2Department of Rehabilitation and Movement Science, University of Medicine and Dentistry of New Jersey, Newark, New Jersey 07107

Keywords: Natural movements, submovements, basal ganglia, force amplitude, change, and duration, proprioceptive error, globus pallidus, subthalamic nucleus.

Mitochondrial Mayhem: The Mitochondrion as a Modulator of Iron Metabolism and its Role in Disease

Antioxidants & Redox Signaling. Online Ahead of Editing: February 27, 2011. doi:10.1089/ars.2011.3921.

Michael Li-Hsuan Huang, Darius John Rowland Lane, Des R. Richardson.
University of Sydney, Pathology, Sydney, New South Wales, Australia.

Keywords: mitochondrion, iron metabolism, mitochondrial ferritin, mitoferrins 1 and 2, ABCBs 6,7 and 10, frataxin, iron storage, iron uptake, heme and iron–sulfur cluster synthesis, regulation of whole cell iron metabolism.

The Minimal Proteome in the Reduced Mitochondrion of the Parasitic Protist Giardia intestinalis

Frataxin is always present in all eukaryotes, it seems essential to life, surprisingly in this paper describes an organism that lacks frataxin, perhaps a more detailed study of this protist would give clues to understand the frataxin function, and help to understand their relationship with the Fe-S clusters.



PLoS ONE 6(2): e17285. doi:10.1371/journal.pone.0017285

Petr L. Jedelský1,2, Pavel Doležal1, Petr Rada1, Jan Pyrih1, Ondřej Šmíd1, Ivan Hrdý1, Miroslava Šedinová1, Michaela Marcinčiková1, Lubomír Voleman1, Andrew J. Perry3, Neritza Campo Beltrán1, Trevor Lithgow3, Jan Tachezy1*

1 Department of Parasitology, Faculty of Science, Charles University in Prague, Prague, Czech Republic, 2 Laboratory of Mass Spectrometry, Faculty of Science, Charles University in Prague, Prague, Czech Republic, 3 Department of Biochemistry & Molecular Biology, Monash University, Clayton Campus, Melbourne, Australia


A striking deviation from other eukaryotes is the absence of frataxin in Giardia mitosomes. Frataxin is invariably present in eukaryotes that contain the ISC-type FeS cluster assembly machinery.

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Lu AA24493 - Expected report data of clinical phase II

Lu AA24493 (novel carbamoylated form of human erythropoietin (EPO)) is in clinical phase II in the treatment of Friedreich's ataxia and in clinical phase I in the treatment of acute ischaemic stroke. Ongoing studies in both programmes are expected to report data in the first half of 2011.

Regulation of cellular iron metabolism

Biochem. J. (2011) 434 (365–381)

Jian Wang*† and Kostas Pantopoulos*†‡1
*Department of Biochemistry, University of Texas Southwestern Medical Center, U.S.A. †Lady Davis Institute for Medical Research, Sir Mortimer B. Davis Jewish General Hospital, Canada.
‡Department of Medicine, McGill University, Canada

Keywords: frataxin, ferritin, ferroportin, iron-regulatory protein 1 (IRP1), iron-regulatory protein 2 (IRP2), iron–sulfur cluster (ISC), transferrin receptor (TfR).

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Patient Reported Outcomes in Friedreich's Ataxia Patients After Withdrawal From Treatment With Idebenone (PROTI)

First Received: February 22, 2011 Last Updated: February 23, 2011

Sponsor: Santhera Pharmaceuticals
Information provided by: Santhera Pharmaceuticals
ClinicalTrials.gov Identifier: NCT01303406

Locations: United Kingdom, The National Hospital, University College London

Principal Investigator: Paola Giunti, M.D, Institute of Neurology, The National Hospital, University College London

The potential application of iron chelators for the treatment of neurodegenerative diseases.

Metallomics. 2011 Feb 22. [Epub ahead of print]

Hider RC, Roy S, Ma YM, Le Kong X, Preston J.

King's College London, Institute of Pharmaceutical Science, London

Keywords: neurodegenerative disease, Alzheimer's disease, Parkinson's disease, Friedreich's ataxia, Hallervorden Spatz syndrome and macular degeneration, redox active metals, copper, iron, selective chelation.

International Study Shows That Reprogramming Cells Leads To Genomic Aberrations

Medical News Today, Article Date: 22 Feb 2011

"It's a discordant note in the symphony of good news that usually accompanies stem cell research announcements."

"The scientists draw the conclusion that reprogrammed stem cells need to be extensively investigated before they can even be considered for use in regenerative medicine."

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First Trial To Compare Autologous Cells With Donor Cells For Dilated Cardiomyopathy

Medical News Today, Article Date: 22 Feb 2011

"complement of trials being offered at UHealth broadens the availability of experimental therapy to patients and offers an alternative to the growing trend of medical tourism for cell based therapies." (University of Miami Miller School of Medicine) Read more ...