Effectiveness and safety of treatments for degenerative ataxias: A systematic review

http://www3.interscience.wiley.com/journal/122371107/abstract?CRETRY=1&SRETRY=0

Effectiveness and safety of treatments for degenerative ataxias: A systematic review

M.Mar Trujillo-Martín, PhD 1 *, Pedro Serrano-Aguilar, MD, PhD 2 3, Fernando Monton-Álvarez, MD, PhD 4, Romen Carrillo-Fumero, PhD 1

1Canary Islands Research and Health Foundation (Fundación Canaria de Investigación y Salud-FUNCIS), Santa Cruz de Tenerife, Canary Islands, Spain 2Planning and Evaluation Unit, Canary Islands Health Authority, Santa Cruz de Tenerife, Canary Islands, Spain 3CIBER Epidemiología y Salud Pública (CIBERESP), Spain 4Department of Neurology, University Hospital of Na Sa de la Candelaria, Santa Cruz de Tenerife, Canary Islands, Spain

email: M.Mar Trujillo-Martín (matrumar@gobiernodecanarias.org)

^*Correspondence to M.Mar Trujillo-Martín, Servicio de Evaluación y Planificación, Servicio Canario de la Salud, Pérez de Rozas, 5-4^a planta, 38004 Santa Cruz de Tenerife, España

Potential conflict of interest: None reported.

Funded by:
Spanish Ministry of Health
Consumption in cooperation
Fundación Canaria de Investigación y Salud (FUNCIS)

Keywords

rare diseases • degenerative ataxia • systematic review • treatment effectiveness • treatment safety

Received: 13 October 2008; Accepted: 3 March 2009

Digital Object Identifier (DOI)

Evidence That Phosphorylation of Iron Regulatory Protein 1 at Serine 138 Destabilizes the [4Fe-4S] Cluster in Cytosolic Aconitase by Enhancing 4Fe-3Fe

Originally published In Press as doi:10.1074/jbc.M807717200 on March 6, 2009 J. Biol. Chem., Vol. 284, Issue 19, 12701-12709, May 8, 2009

Evidence That Phosphorylation of Iron Regulatory Protein 1 at Serine 138 Destabilizes the [4Fe-4S] Cluster in Cytosolic Aconitase by Enhancing 4Fe-3Fe Cycling^*

Kathryn M. Deck, Aparna Vasanthakumar, Sheila A. Anderson, Jeremy B. Goforth¹, M. Claire Kennedy, William E. Antholine^¶, and Richard S. Eisenstein²

From the Department of Nutritional Sciences, University of Wisconsin-Madison, Madison, Wisconsin 53706, the Biochemistry Department, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, and the ^¶National Biomedical ESR Center, Department of Biophysics, Medical College of Wisconsin, Milwaukee, Wisconsin 53226-3548

Received for publication, October 6, 2008 , and in revised form, March 5, 2009.

This paper is dedicated to the memory of Helmut Beinert.

^* This work was supported in part by National Institutes of Health Grants DK 66600 (to R. S. E.) and National Biomedical ESR Center Grant EB001980. This work was also supported by United States Department of Agriculture Cooperative States Research Education and Extension Service Grant 2006-35200-16604 and University of Wisconsin-Madison Hatch Project 4885 (to R. S. E.).

The on-line version of this article (available at http://www.jbc.org/) contains supplemental Figs. S1 and S2.

¹ Supported by National Institutes of Health Training Grant T32 DK007665.

² To whom correspondence should be addressed: University of Wisconsin, Dept. of Nutritional Sciences, 1415 Linden Dr., Madison, WI 53706. Tel.: 608-262-5830; Fax: 608-262-5860; E-mail: eisenste@nutrisci.wisc.edu
.

PGC-1α-induced improvements in skeletal muscle metabolism and insulin sensitivity

Appl. Physiol. Nutr. Metab. 34(3): 307–314 (2009) doi:10.1139/H09-008 Published by NRC Research Press / Publié par les Presses scientifiques du CNRC

PGC-1α-induced improvements in skeletal muscle metabolism and insulin sensitivity

Arend Bonen

CARACTERIZACIÓN Y ANÁLISIS FUNCIONAL DE UNA FAMILIA

Full text: http://www.tesisenred.net/TESIS_UdL/AVAILABLE/TDX-0204105-125138//Tmrm01de04.pdf

CARACTERIZACIÓN Y ANÁLISIS FUNCIONAL DE UNA FAMILIADE GLUTAREDOXINAS MONOTIÓLICAS EN LA LEVADURA Saccharomyces cerevisiae

Teresa Rodríguez-Manzaneque Martínez Julio 2002

Cluster Fe-S-Frataxin

Evolution based on domain combinations: the case of glutaredoxins

Full text: http://www.biomedcentral.com/content/pdf/1471-2148-9-66.pdf


Evolution based on domain combinations: the case of glutaredoxin

BMC Evolutionary Biology 2009, 9:66 doi:10.1186/1471-2148-9-66
Rui Alves (ralves@cmb.udl.es)
Ester Vilapinyo (evilaprinyo@cmb.udl.es)
Albert Sorribas (albert.sorribas@cmb.udl.es)
Enrique Herrero (enric.herrero@cmb.udl.es

PGC-1/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders

Human Molecular Genetics Advance Access originally published online on March 18, 2009
Human Molecular Genetics 2009 18(10):1805-1812; doi:10.1093/hmg/ddp093

PGC-1/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders

Sarika Srivastava1,,, Francisca Diaz1,, Luisa Iommarini1,3, Karine Aure4, Anne Lombes4 and Carlos T. Moraes1,2,*
1 Department of Neurology 2 Department of Cell Biology and Anatomy, University of Miami School of Medicine, 1095 NW 14th Terrace, Miami, FL 33136, USA 3 Dipartimento di Scienze Neurologiche, Università di Bologna, Bologna, Italy 4 Inserm 582; UPMC-Paris6; AP/HP, Paris F-75013, France
* To whom correspondence should be addressed. Tel: +1 3052435858; Fax: +1 3052433914; Email: cmoraes@med.miami.edu

Present address: Harvard Medical School, Boston, MA 02115, USA.

Resveratrol Highlights

http://www.lmcfood.dk/fileadmin/lmc_files/food/Kristensen-5_Resveratrol-Cell-2006.pdf

Lagouge et al., Resveratrol Improves Mitochondrial Function and Protects against Metabolic Disease by Activating SIRT1 and PGC-1a,
Cell (2006), doi:10.1016/j.cell.2006.11.013

Resveratrol Improves Mitochondrial Function and Protects against Metabolic Disease by Activating SIRT1 and PGC-1a

Marie Lagouge,8,1 Carmen Argmann,8,1 Zachary Gerhart-Hines,2 Hamid Meziane,3 Carles Lerin,2
Frederic Daussin,4 Nadia Messadeq,3 Jill Milne,5 Philip Lambert,5 Peter Elliott,5 Bernard Geny,4 Markku Laakso,6
Pere Puigserver,2 and Johan Auwerx1,3,7,* 1 Institut de Ge´ ne´ tique et de Biologie Mole´ culaire et Cellulaire, CNRS / INSERM / ULP, 67404 Illkirch, France
2Department of Cell Biology, John Hopkins University School of Medicine, Baltimore, MD 21205, USA
3 Institut Clinique de la Souris, BP10142, 67404, Illkirch, France
4Department of Respiratory, Cardiocirculatory and Exercise Physiology, Hoˆ pitaux Universitaires, 67000 Strasbourg, France
5 Sirtris Pharmaceutical, Cambridge, MA 02139, USA
6Department of Medicine, University of Kuopio, 70211 Kuopio, Finland
7IGBMC-ICS, 67404 Illkirch, France
8These authors contributed equally to this work.

*Contact: auwerx@igbmc.u-strasbg.fr

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http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowPDF&ArtikelNr=85386&Ausgabe=230907&ProduktNr=224154&filename=85386.pdf

Neurosignals 2005;14:61–70
DOI: 10.1159/000085386
Received: December 6, 2004
Accepted after revision: March 1, 2005

Unique Properties of Polyphenol Stilbenes in the Brain: More than Direct Antioxidant Actions; Gene/Protein Regulatory Activity

Sylvain Doré
Johns Hopkins University, School of Medicine, Baltimore, Md. , USA

________________________________

http://www.ncbi.nlm.nih.gov/pubmed/18629638?dopt=Abstract

Neurochem Res. 2008 Dec;33(12):2444-71. Epub 2008 Jul 16.

Cellular stress response: a novel target for chemoprevention and nutritional neuroprotection in aging, neurodegenerative disorders and longevity.

Calabrese V, Cornelius C, Mancuso C, Pennisi G, Calafato S, Bellia F, Bates TE, Giuffrida Stella AM, Schapira T, Dinkova Kostova AT, Rizzarelli E.Section of Biochemistry and Molecular Biology, Department of Chemistry, Faculty of Medicine, University of Catania, Viale Andrea Doria 6, 95100, Catania, Italy. calabres@unict.it


_________________________________


http://www3.interscience.wiley.com/journal/121682622/abstract?CRETRY=1&SRETRY=0

Histone Deacetylase Inhibition Activity and Molecular Docking of (E )-Resveratrol: Its Therapeutic Potential in Spinal Muscular Atrophy

Didem Dayangaç-Erden 1,*, Gamze Bora 1 , Peruze Ayhan 2 , Çetin Kocaefe 1 , Sevim Dalkara 3 , Kemal Yelekçi 4 , Ayhan S. Demir 2 and Hayat Erdem-Yurter 1
1 Department of Medical Biology, Faculty of Medicine, Hacettepe University, Ankara, Turkey 2 Department of Chemistry, Middle East Technical University, Ankara, Turkey 3 Department of Pharmaceutical Chemistry, Faculty of Pharmacy, Hacettepe University, Ankara, Turkey 4 Department of Statistics and Computer Sciences, Faculty of Arts and Science, Kadir Has University, Istanbul, Turkey
Correspondence to *Dr Didem Dayangaç-Erden, didayan@hacettepe.edu.tr
Copyright Journal compilation © 2009 Blackwell Munksgaard
KEYWORDS
(E )-resveratrol • molecular docking • SMN2 • spinal muscular atrophy

________________________________



http://ntp.niehs.nih.gov/ntp/htdocs/Chem_Background/ExSumPDF/resveratrol.pdf

Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPAR{gamma} pathway as a therapeutic target

Hum Mol Genet. 2009 Apr 17. [Epub ahead of print]
Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPAR{gamma} pathway as a therapeutic target in Friedreich's ataxia.
Coppola G, Marmolino D, Lu D, Wang Q, Cnop M, Rai M, Acquaviva F, Cocozza S, Pandolfo M, Geschwind DH.
Program in Neurogenetics, Department of Neurology David Geffen School of Medicine, University of California at Los Angeles, CA 90095.

OPEN ACCESS:

http://hmg.oxfordjournals.org/cgi/content/full/18/13/2452

http://hmg.oxfordjournals.org/cgi/reprint/18/13/2452.pdf

Brain iron homeostasis and neurodegenerative disease

Citation:

Neurology, Vol. 72, Issue 16, 1436-1440, April 21, 2009
CLINICAL IMPLICATIONS OF NEUROSCIENCE RESEARCH

Brain iron homeostasis and neurodegenerative disease
Eduardo E. Benarroch

Article topics:
Physiology..Transferrin
Medicine..Genes
Medicine..Iron Metabolism
Cell Biology..Endothelial Cells
Physiology..Homeostasis
Medicine..Gene Expression
Medicine..Genetics
Medicine..Neurodegenerative Diseases
Medicine..Cardiovascular Genetics
Biochemistry..Ferritin
Medicine..Friedreich Ataxia
Biochemistry..Pantothenate Kinase
Cell Biology..Astrocytes
Biochemistry..Kinases
Medicine..Ataxia

http://www.neurology.org/cgi/content/citation/72/16/1436?rss=1

E. coli mismatch repair acts downstream of replication fork stalling to stabilize the expanded (GAA.TTC)(n) sequence.

Mutat Res.Author manuscript; available in PMC 2009 February 10.
Published in final edited form as:
Mutat Res. 2009 February 10; 661(1-2): 71–77.
Published online 2008 November 13. doi: 10.1016/j.mrfmmm.2008.11.003. PMCID: PMC2637364
NIHMSID: NIHMS83510

Copyright notice and Disclaimer
E. coli mismatch repair acts downstream of replication fork stalling to stabilize the expanded (GAA·TTC)n sequence
Rebecka L. Bourn,1 Paul M. Rindler,1 Laura M. Pollard,1 and Sanjay I. Bidichandani1,2
1Department of Biochemistry & Molecular Biology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA
2Department of Pediatrics, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA
Address all correspondence and requests for reprints to: Sanjay I. Bidichandani, MBBS, PhD, 975 NE 10th St., BRC458, Oklahoma City, OK 73104, USA, Tel: (405) 271-1360, Fax: (405) 271-3910, Email: Sanjay-Bidichandani@ouhsc.edu
The publisher's final edited version of this article is available at Mutat Res.


Keywords: mismatch repair, triplet repeat instability, GAA triplet repeat, replication fork stalling