NCBI, Public on Jul 02, 2010
Experiment type: Expression profiling by array
Keywords: Friedreich’s ataxia (FRDA), GAA•TTC trinucleotide repeats, FXN gene, induced pluripotent stem cells (iPSCs), fibroblasts, retroviral transduction, Msh2, repeat instability.
Wednesday, August 11, 2010
A Phase 3, Double-blind, Placebo-Controlled Trial of Idebenone in Friedreich Ataxia
Arch Neurol. 2010;67(8):941-947. doi:10.1001/archneurol.2010.168
David R. Lynch, MD, PhD ; Susan L. Perlman, MD ; Thomas Meier, PhD
Monday, August 9, 2010
Automated quantitative gait analysis in animal models of movement disorders
BMC Neuroscience 2010, 11:92doi:10.1186/1471-2202-11-92
Caroline Vandeputte, Jean-Marc Taymans, Cindy Casteels, Frea Coun, Yicheng Ni, Koen Van Laere and Veerle Baekelandt.
OPEN ACCESS
Conclusion
The automated quantitative gait analysis test may be a useful tool to study both motor impairment and recovery associated with various neurological motor disorders.
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Caroline Vandeputte, Jean-Marc Taymans, Cindy Casteels, Frea Coun, Yicheng Ni, Koen Van Laere and Veerle Baekelandt.
OPEN ACCESS
Conclusion
The automated quantitative gait analysis test may be a useful tool to study both motor impairment and recovery associated with various neurological motor disorders.
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Erythropoiesis and iron sulfur cluster biogenesis
Advances in Hematology, Received 1 March 2010; Revised 4 June 2010; Accepted 2 August 2010
Hong Ye and Tracey A. Rouault
OPEN ACCES
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Hong Ye and Tracey A. Rouault
OPEN ACCES
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Wednesday, August 4, 2010
Aluminum induces neurodegeneration and its toxicity arises from increased iron accumulation and reactive oxygen species (ROS) production.
Neurobiol Aging. 2010 Jul 29.
Wu Z, Du Y, Xue H, Wu Y, Zhou B.
State Key Laboratory of Biomembrane and Membrane Biotechnology, School of Life Sciences, Tsinghua University, Beijing 100084, China.
I would like to emphasize: .../... "suggesting Friedreich's ataxia patients might be more susceptible to Al toxicity".../...
Wu Z, Du Y, Xue H, Wu Y, Zhou B.
State Key Laboratory of Biomembrane and Membrane Biotechnology, School of Life Sciences, Tsinghua University, Beijing 100084, China.
I would like to emphasize: .../... "suggesting Friedreich's ataxia patients might be more susceptible to Al toxicity".../...
A rapid, noninvasive immunoassay for frataxin: Utility in assessment of Friedreich ataxia.
Mol Genet Metab. 2010 Jul 8. [Epub ahead of print]
Deutsch EC, Santani AB, Perlman SL, Farmer JM, Stolle CA, Marusich MF, Lynch DR.
Department of Pharmacology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Keywords: Friedreich ataxia (FRDA), frataxin, Western blot analysis, fibroblasts, lymphocytes, , muscle biopsies, lateral flow immunoassay, buccal cells.
Deutsch EC, Santani AB, Perlman SL, Farmer JM, Stolle CA, Marusich MF, Lynch DR.
Department of Pharmacology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Keywords: Friedreich ataxia (FRDA), frataxin, Western blot analysis, fibroblasts, lymphocytes, , muscle biopsies, lateral flow immunoassay, buccal cells.
Saturday, July 31, 2010
Current and emerging treatment options in the management of Friedreich ataxia
Neuropsychiatric Disease and Treatment, Published Date July 2010 , Volume 2010:6 Pages 491 - 499
Michelangelo Mancuso, Daniele Orsucci, Anna Choub, Gabriele Siciliano
Department of Neuroscience, Neurological Clinic, University of Pisa, Pisa, Italy
Keywords: Friedreich ataxia (FRDA), Oxidative damage, mitochondria, antioxidant protection, idebenone, cardiac hypertrophy, frataxin gene transcription, iron-chelating therapies, erythropoietin, histone deacetylase inhibitors, gene-based strategies.
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Michelangelo Mancuso, Daniele Orsucci, Anna Choub, Gabriele Siciliano
Department of Neuroscience, Neurological Clinic, University of Pisa, Pisa, Italy
Keywords: Friedreich ataxia (FRDA), Oxidative damage, mitochondria, antioxidant protection, idebenone, cardiac hypertrophy, frataxin gene transcription, iron-chelating therapies, erythropoietin, histone deacetylase inhibitors, gene-based strategies.
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EMBL scientists identify proteins that ensure iron balance
EurekAlert, Public release date: 4-Aug-2010
MEASURING LEVELS OF FRATAXIN
Patent about methods and materials involved in measuring levels of a frataxin polypeptide present in a differents biological samples.
MAYO FOUNDATION FOR MEDICAL EDUCATION AND RESEARCH, OGLESBEE, Devin, MATERN, Dietrich, ISAYA, Grazia.
MAYO FOUNDATION FOR MEDICAL EDUCATION AND RESEARCH, OGLESBEE, Devin, MATERN, Dietrich, ISAYA, Grazia.
Friday, July 30, 2010
Adaptive robot training for the treatment of incoordination in Multiple Sclerosis
Journal of NeuroEngineering and Rehabilitation 2010, 7:37doi:10.1186/1743-0003-7-37
Elena Vergaro, Valentina Squeri, Giampaolo Brichetto, Maura Casadio, Pietro Morasso, Claudio Solaro and Vittorio Sanguineti.
OPEN ACCES
Abstract
Background
Cerebellar symptoms are extremely disabling and are common in Multiple Sclerosis (MS) patients. In this feasibility study, we developed and tested a robot therapy protocol, aimed at the rehabilitation of incoordination in MS subjects.
Methods
Eight subjects with clinically defined MS performed planar reaching movements while grasping the handle of a robotic manipulandum, which generated forces that either reduced (error-reducing, ER) or enhanced (error-enhancing, EE) the curvature of their movements, assessed at the beginning of each session. The protocol was designed to adapt to the individual subjects' impairments, as well as to improvements between sessions (if any). Each subject went through a total of eight training sessions. To compare the effect of the two variants of the training protocol (ER and EE), we used a cross-over design consisting into two blocks of sessions (four ER and four EE; 2 sessions/week), separated by a 2-week wash-out period. The order of application of ER and EE exercises was randomized across subjects. The primary outcome measure was the modification of the Nine Hole Peg Test (NHPT) score. Other clinical scales and movement kinematics were taken as secondary outcomes.
Results
Most subjects revealed a preserved ability to adapt to the robot-generated forces. No significant differences were observed in EE and ER training. However over sessions, subjects exhibited an average 24 % decrease in their NHPT score. The other clinical scales showed small improvements for at least some of the subjects. After training, movements became smoother, and their curvature decreased significantly over sessions.
Conclusions
The results point to an improved coordination over sessions, and suggest a potential benefit of a short-term, customized, and adaptive robot therapy for MS subjects.
FULL TEXT PDF
Elena Vergaro, Valentina Squeri, Giampaolo Brichetto, Maura Casadio, Pietro Morasso, Claudio Solaro and Vittorio Sanguineti.
OPEN ACCES
Abstract
Background
Cerebellar symptoms are extremely disabling and are common in Multiple Sclerosis (MS) patients. In this feasibility study, we developed and tested a robot therapy protocol, aimed at the rehabilitation of incoordination in MS subjects.
Methods
Eight subjects with clinically defined MS performed planar reaching movements while grasping the handle of a robotic manipulandum, which generated forces that either reduced (error-reducing, ER) or enhanced (error-enhancing, EE) the curvature of their movements, assessed at the beginning of each session. The protocol was designed to adapt to the individual subjects' impairments, as well as to improvements between sessions (if any). Each subject went through a total of eight training sessions. To compare the effect of the two variants of the training protocol (ER and EE), we used a cross-over design consisting into two blocks of sessions (four ER and four EE; 2 sessions/week), separated by a 2-week wash-out period. The order of application of ER and EE exercises was randomized across subjects. The primary outcome measure was the modification of the Nine Hole Peg Test (NHPT) score. Other clinical scales and movement kinematics were taken as secondary outcomes.
Results
Most subjects revealed a preserved ability to adapt to the robot-generated forces. No significant differences were observed in EE and ER training. However over sessions, subjects exhibited an average 24 % decrease in their NHPT score. The other clinical scales showed small improvements for at least some of the subjects. After training, movements became smoother, and their curvature decreased significantly over sessions.
Conclusions
The results point to an improved coordination over sessions, and suggest a potential benefit of a short-term, customized, and adaptive robot therapy for MS subjects.
FULL TEXT PDF
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