Movement Disorders, Early View (Articles online in advance of print),Published Online: 11 Dec 2009
Arunjot Singh, MS, Elizabeth Epstein, BA, Lauren M. Myers, BA, Jennifer M. Farmer, MS, David R. Lynch, MD, PhD
Departments of Neurology and Pediatrics, University of Pennsylvania School of Medicine and The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
Keywords: ataxia, sensory neuron, dysarthria, balance, clinical trial, Friedreich Ataxia (FA), speech, therapeutic monitoring.
Sunday, December 13, 2009
AF4 Is a Critical Regulator of the IGF-1 Signaling Pathway during Purkinje Cell Development
The Journal of Neuroscience, December 9, 2009, 29(49):15366-15374; doi:10.1523/JNEUROSCI.5188-09.2009
Emmanuelle Bitoun,1,2 * Mattéa J. Finelli,1,2 * Peter L. Oliver,1,2 Sheena Lee,1 and Kay E. Davies1,2
1Medical Research Council Functional Genomics Unit, and 2Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, United Kingdom
Keywords: insulin-like growth factor 1 (IGF-1), cerebellar ataxia, neuronal cell death, targeted for therapy, Purkinje cell (PC) death, cerebellum, transcriptional cofactor.
1Medical Research Council Functional Genomics Unit, and 2Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, United Kingdom
Keywords: insulin-like growth factor 1 (IGF-1), cerebellar ataxia, neuronal cell death, targeted for therapy, Purkinje cell (PC) death, cerebellum, transcriptional cofactor.
Friday, December 11, 2009
Novel Synchrotron-Based Analyses of Metal Pathology in Friedreich’s Ataxia
University of Saskatchewan Library, Electronic Theses & Dissertations
Document Type Thesis
Popescu, Bogdan Florin GH.
Keywords: Copper, Iron, Zinc, Rapid Scanning X-ray Fluorescence Mapping, Synchrotron, Brain, Metals, Neurodegeneration, Friedreich's ataxia.
Full text pdf
Document Type Thesis
Popescu, Bogdan Florin GH.
Keywords: Copper, Iron, Zinc, Rapid Scanning X-ray Fluorescence Mapping, Synchrotron, Brain, Metals, Neurodegeneration, Friedreich's ataxia.
Full text pdf
Thursday, December 10, 2009
The ins and outs of mitochondrial iron-loading: the metabolic defect in Friedreich's ataxia.
J Mol Med. 2009 Dec 9.
Richardson DR, Huang ML, Whitnall M, Becker EM, Ponka P, Suryo Rahmanto Y.
Iron Metabolism and Chelation Program, Department of Pathology and Bosch Institute, Blackburn Building (D06), University of Sydney, Sydney, NSW, 2006, Australia, d.richardson@med.usyd.edu.au.
Keywords: Friedreich's ataxia, cardio- and neurodegenerative disease, frataxin, mitochondrial iron-overload, transferrin receptor-1 upregulation, ferritin, ferroportin1, mitoferrin2, iron-sulfur cluster (ISC), mitochondrial ferritin, compensatory alterations.
Richardson DR, Huang ML, Whitnall M, Becker EM, Ponka P, Suryo Rahmanto Y.
Iron Metabolism and Chelation Program, Department of Pathology and Bosch Institute, Blackburn Building (D06), University of Sydney, Sydney, NSW, 2006, Australia, d.richardson@med.usyd.edu.au.
Keywords: Friedreich's ataxia, cardio- and neurodegenerative disease, frataxin, mitochondrial iron-overload, transferrin receptor-1 upregulation, ferritin, ferroportin1, mitoferrin2, iron-sulfur cluster (ISC), mitochondrial ferritin, compensatory alterations.
Altered gene expression and DNA damage in peripheral blood cells from Friedreich's ataxia patients
Gene Expression Omnibus, Series GSE11204
Experiment type Expression profiling by array, Public on Dec 08, 2009
Keywords: Friedreich's ataxia; frataxin; mitochondrial DNA damage; nuclear DNA damage; genotoxic stress
Experiment type Expression profiling by array, Public on Dec 08, 2009
Keywords: Friedreich's ataxia; frataxin; mitochondrial DNA damage; nuclear DNA damage; genotoxic stress
Wednesday, December 9, 2009
A Wireless Brain-Machine Interface for Real-Time Speech Synthesis
"Perhaps the most debilitating aspect of profound paralysis due to accident, stroke, or disease is loss of the ability to speak. The loss of speech not only makes the communication of needs to caregivers very difficult, but it also leads to profound social isolation of the affected individual."
PLoS ONE 4(12): e8218. doi:10.1371/journal.pone.0008218
OPEN ACCESS
Frank H. Guenther1,2*, Jonathan S. Brumberg1,3, E. Joseph Wright3, Alfonso Nieto-Castanon4, Jason A. Tourville1, Mikhail Panko1, Robert Law1, Steven A. Siebert3, Jess L. Bartels3, Dinal S. Andreasen3,5, Princewill Ehirim6, Hui Mao7, Philip R. Kennedy3
1 Department of Cognitive and Neural Systems and Sargent College of Health and Rehabilitation Sciences, Boston University, Boston, Massachusetts, United States of America, 2 Division of Health Sciences and Technology, Harvard University-Massachusetts Institute of Technology, Cambridge, Massachusetts, United States of America, 3 Neural Signals Inc., Duluth, Georgia, United States of America, 4 StatsANC LLC, Buenos Aires, Argentina, 5 Georgia Tech Research Institute, Marietta, Georgia, United States of America, 6 Gwinnett Medical Center, Lawrenceville, Georgia, United States of America, 7 Emory Center for Systems Imaging, Emory University Hospital, Atlanta, Georgia, United States of America
Abstract
Background: Brain-machine interfaces (BMIs) involving electrodes implanted into the human cerebral cortex have recently been developed in an attempt to restore function to profoundly paralyzed individuals. Current BMIs for restoring communication can provide important capabilities via a typing process, but unfortunately they are only capable of slow communication rates. In the current study we use a novel approach to speech restoration in which we decode continuous auditory parameters for a real-time speech synthesizer from neuronal activity in motor cortex during attempted speech.
Methodology/Principal Findings: Neural signals recorded by a Neurotrophic Electrode implanted in a speech-related region of the left precentral gyrus of a human volunteer suffering from locked-in syndrome, characterized by near-total paralysis with spared cognition, were transmitted wirelessly across the scalp and used to drive a speech synthesizer. A Kalman filter-based decoder translated the neural signals generated during attempted speech into continuous parameters for controlling a synthesizer that provided immediate (within 50 ms) auditory feedback of the decoded sound. Accuracy of the volunteer's vowel productions with the synthesizer improved quickly with practice, with a 25% improvement in average hit rate (from 45% to 70%) and 46% decrease in average endpoint error from the first to the last block of a three-vowel task.
Conclusions/Significance: Our results support the feasibility of neural prostheses that may have the potential to provide near-conversational synthetic speech output for individuals with severely impaired speech motor control. They also provide an initial glimpse into the functional properties of neurons in speech motor cortical areas.
Full text pdf
PLoS ONE 4(12): e8218. doi:10.1371/journal.pone.0008218
OPEN ACCESS
Frank H. Guenther1,2*, Jonathan S. Brumberg1,3, E. Joseph Wright3, Alfonso Nieto-Castanon4, Jason A. Tourville1, Mikhail Panko1, Robert Law1, Steven A. Siebert3, Jess L. Bartels3, Dinal S. Andreasen3,5, Princewill Ehirim6, Hui Mao7, Philip R. Kennedy3
1 Department of Cognitive and Neural Systems and Sargent College of Health and Rehabilitation Sciences, Boston University, Boston, Massachusetts, United States of America, 2 Division of Health Sciences and Technology, Harvard University-Massachusetts Institute of Technology, Cambridge, Massachusetts, United States of America, 3 Neural Signals Inc., Duluth, Georgia, United States of America, 4 StatsANC LLC, Buenos Aires, Argentina, 5 Georgia Tech Research Institute, Marietta, Georgia, United States of America, 6 Gwinnett Medical Center, Lawrenceville, Georgia, United States of America, 7 Emory Center for Systems Imaging, Emory University Hospital, Atlanta, Georgia, United States of America
Abstract
Background: Brain-machine interfaces (BMIs) involving electrodes implanted into the human cerebral cortex have recently been developed in an attempt to restore function to profoundly paralyzed individuals. Current BMIs for restoring communication can provide important capabilities via a typing process, but unfortunately they are only capable of slow communication rates. In the current study we use a novel approach to speech restoration in which we decode continuous auditory parameters for a real-time speech synthesizer from neuronal activity in motor cortex during attempted speech.
Methodology/Principal Findings: Neural signals recorded by a Neurotrophic Electrode implanted in a speech-related region of the left precentral gyrus of a human volunteer suffering from locked-in syndrome, characterized by near-total paralysis with spared cognition, were transmitted wirelessly across the scalp and used to drive a speech synthesizer. A Kalman filter-based decoder translated the neural signals generated during attempted speech into continuous parameters for controlling a synthesizer that provided immediate (within 50 ms) auditory feedback of the decoded sound. Accuracy of the volunteer's vowel productions with the synthesizer improved quickly with practice, with a 25% improvement in average hit rate (from 45% to 70%) and 46% decrease in average endpoint error from the first to the last block of a three-vowel task.
Conclusions/Significance: Our results support the feasibility of neural prostheses that may have the potential to provide near-conversational synthetic speech output for individuals with severely impaired speech motor control. They also provide an initial glimpse into the functional properties of neurons in speech motor cortical areas.
Full text pdf
Supplementation with the reduced form of Coenzyme Q10 decelerates phenotypic characteristics of senescence and induces a peroxisome proliferator-activated receptor- gene expression signature in SAMP1 mice
Molecular Nutrition & Food Research
Received: 4 April 2009; Revised: 10 June 2009; Accepted: 30 June 2009.
Constance Schmelzer 1, Hiroshi Kubo 2, Masayuki Mori 3, Jinko Sawashita 3, Mitsuaki Kitano 2, Kazunori Hosoe 4, Inka Boomgaarden 1, Frank Döring 1 *, Keiichi Higuchi 3
1Institute of Human Nutrition and Food Science, Molecular Prevention, Christian-Albrechts-University of Kiel, Kiel, Germany
2Kaneka Corporation, Frontier Biochemical and Medical Research Laboratories, Takasago, Hyogo, Japan
3Department of Aging Biology, Institute on Aging and Adaptation, Shinshu University Graduate School of Medicine, Matsumoto, Japan
4Kaneka Corporation, Functional Food Ingredients Division, Osaka, Japan
Keywords: CoQ10, gene expression, inflammation, lipid metabolism, peroxisome proliferator-activated receptor- alpha, reduced form (Q10H2) coenzyme Q10 (CoQ10), liver, heart, brain, kidney, stronger impact on gene expression, bioavailability, degenerative processes.
Received: 4 April 2009; Revised: 10 June 2009; Accepted: 30 June 2009.
Constance Schmelzer 1, Hiroshi Kubo 2, Masayuki Mori 3, Jinko Sawashita 3, Mitsuaki Kitano 2, Kazunori Hosoe 4, Inka Boomgaarden 1, Frank Döring 1 *, Keiichi Higuchi 3
1Institute of Human Nutrition and Food Science, Molecular Prevention, Christian-Albrechts-University of Kiel, Kiel, Germany
2Kaneka Corporation, Frontier Biochemical and Medical Research Laboratories, Takasago, Hyogo, Japan
3Department of Aging Biology, Institute on Aging and Adaptation, Shinshu University Graduate School of Medicine, Matsumoto, Japan
4Kaneka Corporation, Functional Food Ingredients Division, Osaka, Japan
Keywords: CoQ10, gene expression, inflammation, lipid metabolism, peroxisome proliferator-activated receptor- alpha, reduced form (Q10H2) coenzyme Q10 (CoQ10), liver, heart, brain, kidney, stronger impact on gene expression, bioavailability, degenerative processes.
Monday, December 7, 2009
Friedreich Ataxia: An Update on Animal Models, Frataxin Function and Therapies
Advances in experimental medicine and biology, ISSN 0065-2598 CODEN AEMBAP. 2009, vol. 652, pp. 247-261 [15 page(s) (article)
GONZALEZ-CABO Pilar ; VICENTE LLORENS José ; PALAU Francesc ; MOLTO Maria Dolores ;
Keywords: Friedreich ataxia ; Frataxin ; Mitochondria ; Iron-sulfur clusters ; Oxidative stress ; Oxidative phosphorylation ; Antioxidant therapy ; Iron chelators ; Recombinant human erythropoietin ; Histone deacetylase inhibitors ;
GONZALEZ-CABO Pilar ; VICENTE LLORENS José ; PALAU Francesc ; MOLTO Maria Dolores ;
Keywords: Friedreich ataxia ; Frataxin ; Mitochondria ; Iron-sulfur clusters ; Oxidative stress ; Oxidative phosphorylation ; Antioxidant therapy ; Iron chelators ; Recombinant human erythropoietin ; Histone deacetylase inhibitors ;
Sunday, December 6, 2009
PGC-1 activation as a therapeutic approach in mitochondrial disease
IUBMB IUBMB Life, 61(11): 1051-1062, 2009
Tina Wenz.
Department of Neurology, University of Miami School of Medicine, Miami, FL 33136, USA
Keywords: Mitochondria, cellular homeostasis, mitochondrial dysfunction, ATP, OXPHOS, PGC-1
.
Tina Wenz.
Department of Neurology, University of Miami School of Medicine, Miami, FL 33136, USA
Keywords: Mitochondria, cellular homeostasis, mitochondrial dysfunction, ATP, OXPHOS, PGC-1
.
Friday, December 4, 2009
Impairment in motor reprogramming in Friedreich ataxia reflecting possible cerebellar dysfunction.
J Neurol. 2009 Dec 3
Keywords: cerebellar and spinocerebellar dysfunction, Friedreich ataxia (FRDA), motor function, cortical pathology, cerebello-cortical projections, , mov6ement execution, movement preparation, age of onset.
Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Royal Children's Hospital, 10th Floor, Flemington Road, Parkville, VIC, 3052, Australia.
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