Characterising the Neuropathology and Neurobehavioural Phenotype in Friedreich Ataxia: A Systematic Review

Characterising the Neuropathology and Neurobehavioural Phenotype in Friedreich Ataxia: A Systematic Review. Louise A. Corben, Nellie Georgiou‑Karistianis, John L. Bradshaw, Marguerite V. Evans‑Galea, Andrew J. Churchyard and Martin B. Delatycki;
From the book Tandem Repeat Polymorphisms: Genetic Plasticity, Neural Diversity and Disease, Edited by: Anthony J. Hannan, ISBN: 978-1-4614-5433-5, Publication date: October 26, 2012

Neuroanatomical Correlates of Depression in Friedreich's Ataxia: a Voxel-Based Morphometry Study.

Neuroanatomical Correlates of Depression in Friedreich's Ataxia: a Voxel-Based Morphometry Study. da Silva CB, Yasuda CL, D'Abreu A, Cendes F, Lopes-Cendes I, França MC Jr.; Cerebellum. 2012 Oct 24.

Keywords: Affective disorders, neurodegenerative diseases, poor quality of life, depression, Beck Depression Inventory, cerebral 3 T MRI scans, structural abnormalities, white matter, reduction of gray matter volumes.

Letter by Peverill Regarding Article, “The Heart in Friedreich Ataxia: Definition of Cardiomyopathy, Disease Severity, and Correlation With Neurological Symptoms”

Letter by Peverill Regarding Article, “The Heart in Friedreich Ataxia: Definition of Cardiomyopathy, Disease Severity, and Correlation With Neurological Symptoms” , Roger E. Peverill, MB, BS, PhD; Circulation. 2012; 126: e272 doi: 10.1161/CIRCULATIONAHA.112.115287

Keywords: Friedreich ataxia (FA), cardiac disease, premature mortality, cardiomyopathy in FA, left ventricular (LV) mass, interventricular septal thickness.

R- and S-Equol have equivalent cytoprotective effects in Friedreich's Ataxia

R- and S-Equol have equivalent cytoprotective effects in Friedreich's Ataxia, Timothy E Richardson and James W Simpkins, BMC Pharmacology and Toxicology 2012, doi:10.1186/2050-6511-13-12. Published: 22 October 2012

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These preliminary data demonstrate that estrogen and soy-derived equols could have a beneficial effect in delaying the onset and decreasing the severity of symptoms in FRDA patients by an antioxidant mechanism. In addition, these data confirm that the protection seen previously with E2 was indeed unrelated to ER binding.

Novel Therapeutic Challenges in Cerebellar Diseases

Novel Therapeutic Challenges in Cerebellar Diseases. Antoni Matilla Dueñas, Carme Serrano, Yerko Ivanovic, Ramiro Alvarez, Pilar Latorre, and David Genıs. Documentos RIBERMOV (19/10/2012)

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New Project: Developing bone marrow transplant and novel therapeutic vectors to treat Friedreich ataxia

New Project: Developing bone marrow transplant and novel therapeutic vectors to treat Friedreich ataxia.  Professor Martin Delatycki.  Federal Government's National Health and Medical Research Council (NHMRC) funding (Australia).

Chemical and technological delivery systems for idebenone: a review of literature production

Chemical and technological delivery systems for idebenone: a review of literature production. Claudia Carbone, Rosario Pignatello, Teresa Musumeci, and Giovanni Puglisi; Expert Opinion on Drug Delivery, November 2012, Vol. 9, No. 11 : Pages 1377-1392. doi: 10.1517/17425247.2012.724396.

Keywords: antioxidant activity, cyclodextrins, drug delivery systems, lipoamino acids, liposomes, microemulsions, nanoparticles, prodrugs, SLN, solid lipid nanoparticles, Idebenone (IDE).

Reply to Beconi et al.: Oral Administration of the Pimelic Diphenylamide HDAC Inhibitor HDACi 4b Is Unsuitable for Chronic Inhibition of HDAC Activity in the CNS In Vivo

Reply to Beconi et al.: Oral Administration of the Pimelic Diphenylamide HDAC Inhibitor HDACi 4b Is Unsuitable for Chronic Inhibition of HDAC Activity in the CNS In Vivo. Joel M. Gottesfeld and Elizabeth A. Thomas. PLoS ONE 7(9): e44498. doi:10.1371/journal.pone.0044498 Comment posted by joelg on 19 Oct 2012 at 18:39 GMT

Consumers Angry That Genetics Testing Company Has Used Their Data to Obtain a Patent

Consumers Angry That Genetics Testing Company Has Used Their Data to Obtain a Patent. Biotechnology Law Report. October 2012, 31(5): 473-473, doi:10.1089/blr.2012.9711.

Novel Frataxin Isoforms May Contribute to the Pathological Mechanism of Friedreich Ataxia

Novel Frataxin Isoforms May Contribute to the Pathological Mechanism of Friedreich Ataxia. Haiyan Xia1, Yun Cao, Xiaoman Dai, Zvonimir Marelja, Di Zhou, Ran Mo, Sahar Al-Mahdawi, Mark A. Pook, Silke Leimkühler, Tracey A. Rouault, Kuanyu Li; PLoS ONE 7(10): e47847. doi:10.1371/journal.pone.0047847

"Findings highly relevant to understanding the mechanism of tissue-specific pathology in FRDA."

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