Targeting Mitochondrial Dysfunction and Neurodegeneration by Means of Coenzyme Q10 and its Analogues.

Curr Med Chem. 2011 Aug 9.

Orsucci D, Mancuso M, Ienco EC, Logerfo A, Siciliano G.
Department of Neuroscience, Neurological Clinic, University of Pisa, Italy

Keywords: Coenzyme Q10, respiratory chain, antioxidant properties, mitochondrial disorders, reactive oxygen species, idebenone, mitoquinone, Friedreich ataxia, cardiac hypertrophy, improve neurological function.

Friedreich's Ataxia: a review from a cardiology perspective.

Ir J Med Sci. 2011 Aug 7.

Bourke T, Keane D.
Cardiac Arrhythmia Service, St Vincent's University Hospital, Dublin, Ireland

Keywords: Friedreich's Ataxia (FA), morbidity, mortality, premature death, Hypertrophic cardiomyopathy, cardiac examination, ECG, ECHO, left ventricular outflow gradient.

Glutathione: a key component of the cytoplasmic labile iron pool.

Biometals. 2011 Jul 17.

Hider RC, Kong XL.
Institute of Pharmaceutical Science, King's College London

Keywords: cytoplasmic labile iron pool, heme, iron sulfur cluster synthesis, iron(II)glutathione, iron(II), manganese(II),synthesis of Fe-S cluster proteins.

STATegics, Inc. Announces a Grant From Friedreich's Ataxia Research Alliance to Support Its Program to Develop Small Molecule Mimetics of Erythropoietin for the Treatment of Friedreich's Ataxia

MENLO PARK, Calif., Aug. 2, 2011 (GLOBE NEWSWIRE) -- STATegics, Inc. announced today that the Friedreich's Ataxia Research Alliance (FARA) has awarded the Company $152,690 to advance its proprietary small molecule erythropoietin mimetic compounds for the treatment of Friedreich's ataxia (FRDA),read more

The scale for the assessment and rating of ataxia correlates with dysarthria assessment in Friedreich's ataxia

Journal of Neurology, DOI: 10.1007/s00415-011-6192-9

Andreas Eigentler, Johanna Rhomberg, Wolfgang Nachbauer, Irmgard Ritzer, Werner Poewe and Sylvia Boesch

Keywords: Dysarthria, motor functions, Frenchay Dysarthria Assessment, clinical and logopedic methodology, reflexes, palate, tongue, intelligibility.

The role of induced pluripotent stem cells in regenerative medicine: neurodegenerative diseases

Stem Cell Research & Therapy 2011, 2:32 doi:10.1186/scrt73, Published: 28 July 2011

Jun Peng and Xianmin Zeng

Keywords: Alzheimer's disease, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, Friedreich's ataxia, neurodegenerative diseases, no effective clinical therapies, human adult somatic cells, induced pluripotent stem cells (iPSCs), in vitro disease mechanism study, in vivo cell replacement therapy.

A pilot trial of deferiprone for neurodegeneration with brain iron accumulation.

Haematologica. 2011 Jul 26. [Epub ahead of print]

Abbruzzese G, Cossu G, Balocco M, Marchese R, Murgia D, Melis M, Galanello R, Barella S, Matta G, Ruffinengo U, Bonuccelli U, Forni GL.

(Clinicaltrials.gov identifier: NTC00907283.)

Keywords: Deferiprone, Friedreich's ataxia, cerebral iron accumulation, pantothenate kinase-associated neurodegeneration, parkinsonism and focal dystonia, Magnetic resonance imaging, neurological examinations, Chelation treatment.

RNA-mediated transcriptional gene silencing in Friedreich ataxia

Current Opinion in Biotechnology, Volume 22, Supplement 1, September 2011, Page S16, European Biotechnology Congress 2011

Sanjay Bidichandani, Angela Castro and Yogesh Chutake

Department of Biochemistry & Molecular Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA

You can find the abstract in the Strasbourg FARA conference summary.
http://www.curefa.org/_pdf/4thInternationalFAConferenceAbstracts.pdf

Assessment of neurological efficacy of idebenone in pediatric patients with Friedreich's ataxia: data from a 6-month controlled study followed by a 12-month open-label extension study.

J Neurol. 2011 Jul 22. [Epub ahead of print]

Meier T, Perlman SL, Rummey C, Coppard NJ, Lynch DR.
Santhera Pharmaceuticals, Liestal, Switzerland.

Keywords: idebenone, neurological function, ICARS, FARS, neurological rating scales, Friedreich's ataxia (FRDA), open-label extension study (IONIA-E), may offer a therapeutic benefit to pediatric FRDA patients.

A polymorphic miR-155 binding site in AGTR1 is associated with cardiac hypertrophy in Friedreich ataxia.

J Mol Cell Cardiol. 2011 Jul 12.

Kelly M, Bagnall RD, Peverill RE, Donelan L, Corben L, Delatycki MB, Semsarian C.
Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Sydney, New South Wales, Australia; Sydney Medical School, University of Sydney, New South Wales, Australia.

Keywords:Friedreich ataxia (FRDA), expanded GAA trinucleotide repeat,frataxin gene (FXN), mitochondrial iron efflux, sensitivity to oxidative stress, variability in cardiac phenotype, genetic modifying factors, single nucleotide polymorphisms (SNPs), Renin-Angiotensin-Aldosterone system (RAAS), angiotensin-II type-1 receptor (AGTR1), angiotensin-converting enzyme (ACE), ACE2, left ventricular internal diameter in diastole (LVIDd), interventricular septal wall thickness (SWT), left ventricular mass (LVM), diastolic blood pressure, role of RAAS polymorphisms as modifiers of cardiac phenotype.