Saturday, August 8, 2009

Cardiac involvement in Friedreich's ataxia: our experience.

Minerva Cardioangiol. 2009 Apr;57(2):269-70.

Fazio G., Vitello D., D’Angelo L., Visconti C., Trapani R., Fabiano A., Di Gesaro G., Novo G., Novo S.


Not abstrac

Friday, August 7, 2009

Nitrative and Oxidative Stress in Toxicology and Disease

OPEN ACCESS

ToxSci Advance Access published online on August 5, 2009 Toxicological Sciences, doi:10.1093/toxsci/kfp179

Ruth A. Roberts1, Debra L. Laskin2, Charles V. Smith3, Fredika M. Robertson4, Erin M.G. Allen5, Jonathan A. Doorn5 and William Slikker6
1 AstraZeneca R&D Safety Assessment, Alderley Park, UK 2 Department of Pharmacology and Toxicology, Rutgers University, Piscataway, NJ 3 Center for Developmental Therapeutics, Seattle Children's Research Institute, Seattle, WA 4 Experimental Therapeutics, The University of Texas M.D. Anderson Cancer Center, Houston, TX 5 College of Pharmacy, University of Iowa, Iowa City, IA 6 NCTR, USFDA, Jefferson, AR
ruth.roberts@astrazeneca.com


Received June 15, 2009; revision received July 22, 2009; accepted July 24, 2009

Abstract

Persistent inflammation and the generation of reactive oxygen and nitrogen species play pivotal roles in tissue injury during disease pathogenesis and as a reaction to toxicant exposures. The associated oxidative and nitrative stress promote diverse pathologic reactions including neurodegenerative disorders, atherosclerosis, chronic inflammation, cancer, and premature labor and stillbirth. These effects occur via sustained inflammation, cellular proliferation and cytotoxicity and via induction of a proangiogenic environment. For example, exposure to the ubiquitous air pollutant ozone leads to generation of reactive oxygen and nitrogen species in lung macrophages that play a key role in subsequent tissue damage. Similarly, studies indicate that genes involved in regulating oxidative stress are altered by anesthetic treatment resulting in brain injury, most notable during development. In addition to a role in tissue injury in the brain, inflammation and oxidative stress are implicated in Parkinson's disease, a neurodegenerative disease characterized by the loss of dopamine neurons. Recent data suggest a mechanistic link between oxidative stress and elevated levels of DOPAL, a neurotoxin endogenous to dopamine neurons. These findings have significant implications for development of therapeutics and identification of novel biomarkers for PD pathogenesis. Oxidative and nitrative stress is also thought to play a role in creating the pro-inflammatory microenvironment associated with the aggressive phenotype of inflammatory breast cancer. An understanding of fundamental concepts of oxidative and nitrative stress can underpin a rational plan of treatment for diseases and toxicities associated with excessive production of reactive oxygen and nitrogen species.

© The Author 2009. Published by Oxford University Press on behalf of the Society of Toxicology. All rights reserved. For permissions, please email: journals.permissions@oxfordjournals.orgThe online version of this article has been published under an open access model. Users are entitled to use, reproduce, disseminate, or display the open access version of this article for non-commercial purposes provided that: the original authorship is properly and fully attributed; the Journal and Oxford University Press are attributed as the original place of publication with the correct citation details given; if an article is subsequently reproduced or disseminated not in its entirety but only in part or as a derivative work this must be clearly indicated. For commercial re-use, please contact journals.permissions@oxfordjournals.org.

Full text: http://toxsci.oxfordjournals.org/cgi/reprint/kfp179v1

Vision based interface system for hands free control of an intelligent wheelchair

Open Access

Jin Sun Ju , Yunhee Shin and Eun Yi Kim
Journal of NeuroEngineering and Rehabilitation 2009, 6:33doi:10.1186/1743-0003-6-33
Published: 6 August 2009

Abstract (provisional)
Due to the shift of the age structure in today's populations, the necessities for developing the devices or technologies to support them have been increasing. Traditionally, the wheelchair, including powered and manual ones, is the most popular and important rehabilitation assistive device for the disabled and the elderly. However, it is still highly restricted especially for severely disabled. As a solution to this, the Intelligent Wheelchairs have received considerable attention as mobility aids. The purpose of this work is to develop the IW interface for providing more convenient and efficient interface to the people the disability in their limbs. This paper proposes an intelligent wheelchair control system for the people with various disabilities. To facilitate a wide variety of user abilities, the proposed system involves the use of face-inclination and mouth-shape information, where the direction of an IW is determined by the inclination of the user's face, while proceeding and stopping are determined by the shapes of the user's mouth. Our system is composed of electric powered wheelchair, data acquisition board, ultrasonic infra-red sensors, a PC camera, and vision system. Then the vision system to analyze user's gestures is performed by three stages: detector, recognizer, and converter. In the detector, the facial region of the intended user is first obtained using Adaboost, thereafter the mouth region is detected based on edge information. The extracted features are sent to the recognizer, which recognizes the face inclination and mouth shape using statistical analysis and K-means clustering, respectively. These recognition results are then delivered to the converter to control the wheelchair. The advantages of the proposed system include 1) accurate recognition of user's intention with minimal user motion and 2) robustness to a cluttered background and the time-varying illumination. To prove these advantages, the proposed system was tested with 34 users in indoor and outdoor environments and the results were compared with those of other systems, then the results showed that the proposed system has superior performance to other systems in terms of speed and accuracy. Therefore, it is proved that proposed system provided a friendly and convenient interface to the severely disabled peo


Full text: http://www.jneuroengrehab.com/content/pdf/1743-0003-6-33.pdf

Wednesday, August 5, 2009

Ataxias with autosomal, X-chromosomal or maternal inheritance.

Can J Neurol Sci. 2009 Jul;36(4):409-28.

Finsterer J.

Krankenanstalt Rudolfstiftung, Vienna, Austria, Europe.

Keywors: Heredoataxias, genetic disorders, cerebellar syndrome, inheritance, autosomal dominant, autosomal recessive, X-linked, and maternally inherited heredoataxias, Friedreich ataxia.

New clues about vitamin D functions in the nervous system

by: E. Garcion, N. Wionbarbot, C. Monteromenei, F. Berger, D. Wion
Trends in Endocrinology and Metabolism, Vol. 13, No. 3. (01 April 2002), pp. 100-105.

Keywords: 1α,25 dihydroxyvitamin D 3 [1,25-(OH) 2 D 3 ], brain function, nuclear receptor, neurons, glial cells, neurotrophic factors, neurotransmitter synthesis, nitric oxide synthase, increase glutathione levels, brain detoxification pathways, neuroprotective, immunomodulatory effects, neurodegenerative diseases, neuroimmune diseases, glioma cell death, brain tumors.

Other link: http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T3K-45BCJ53-7&_user=10&_rdoc=1&_fmt=&_orig=search&_sort=d&_docanchor=&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=36fe7df09fbacd4b33fe816072a1793d

Abnormal Brain Circuits May Prevent Movement Disorder

ScienceDaily (Aug. 4, 2009)
.../...
Standaert says that although dystonia is a relatively rare disorder, the study has implications for other neurological illnesses, such as Parkinson's, Alzheimer's, and Huntington's diseases; ataxia and muscular dystrophies; and even forms of migraine.
.../...

Source: The Journal of Neuroscience, August 5, 2009, 29(31):9740-9747; doi:10.1523/JNEUROSCI.2300-09.2009

Cerebellothalamocortical Connectivity Regulates Penetrance in Dystonia

Miklos Argyelan,1 * Maren Carbon,1,2,3 * Martin Niethammer,1,2,3 Aziz M. Ulug,1 Henning U. Voss,4 Susan B. Bressman,5 Vijay Dhawan,1,2,3 and David Eidelberg1,2,3

1Center for Neurosciences, The Feinstein Institute for Medical Research, North Shore-Long Island Jewish Health System, Manhasset, New York 11030, Departments of 2Neurology and 3Medicine, North Shore University Hospital and New York University School of Medicine, Manhasset, New York 11030, 4Department of Radiology, Weill Cornell Medical College, New York, New York 10065, and 5Mirken Department of Neurology, Beth Israel Medical Center, New York, New York 10003

Tuesday, August 4, 2009

Deficiency in frataxin homologue YFH1 in the yeast Pichia guilliermondii leads to missregulation of iron acquisition and riboflavin biosynthesis and a

Biometals. 2009 Aug 1.

Deficiency in frataxin homologue YFH1 in the yeast Pichia guilliermondii leads to missregulation of iron acquisition and riboflavin biosynthesis and affects sulfate assimilation.



Pynyaha YV, Boretsky YR, Fedorovych DV, Fayura LR, Levkiv AI, Ubiyvovk VM, Protchenko OV, Philpott CC, Sibirny AA.

Institute of Cell Biology, NAS of Ukraine, Drahomanov Street 14/16, 79005, Lviv, Ukraine.


Keyword: riboflavin, YFH1, frataxin homologue, iron, impaired sulfate assimilation, hypersensitivity to hydrogen peroxide, superoxide dismutase.

Frataxin, an iron taxi on the road to cellular Fe–S bioassembly

Traffic and storage of metal ions, J Biol Inorg Chem (2009) 14 (Suppl 1):S53–S56 (Oral Presentation)
G-08
Frataxin, an iron taxi on the road to cellular Fe–S bioassembly
Timothy L. Stemmler
Department of Biochemistry and Molecular Biology, Wayne State
University, School of Medicine, Detroit, MI 48201 USA.
tstemmle@med.wayne.edu

http://www.springerlink.com/content/8x412685h746427g/fulltext.pdf

Keywords: Friedreich’s ataxia, iron homeostasis, iron overload, oxidative stress, mitochondrial DNA stability, iron–sulfur protein, iron chaperone, acidic residues, Fe–S cluster assembly enzyme (Isu),

Friday, July 31, 2009

Double Blind Placebo-Controlled Phase I/II Clinical Trial of Idebenone in Patients With Primary Progressive Multiple Sclerosis

This study is currently recruiting participants.
Verified by National Institutes of Health Clinical Center (CC), July 2009
First Received: July 30, 2009

Objective: The goal of this study is to assess the safety, therapeutic efficacy and mechanism of action of idebenone in primary-progressive multiple sclerosis (PP-MS) patients

Study Type: Interventional
Study Design: Treatment, Randomized, Double-Blind, Placebo Control, Parallel Assignment, Safety/Efficacy Study

Thursday, July 30, 2009

Scientists discover why we never forget how to ride a bicycle

PhysOrg.com, July 17th, 2009

Their research, published this month in Nature Neuroscience, has identified a key nerve cell in the brain that controls the formation of memories for such as riding a bicycle, skiing or eating with chop sticks.

Keywords: cerebellum, brain, co-ordinated movement, molecular layer interneuron, motor skill, brain-computer interfaces, prosthetic devices.