Structural basis for Fe-S cluster assembly and tRNA thiolation mediated by IscS protein-protein interactions.

PLoS Biol. 2010 Apr 13;8(4):e1000354.

Rong Shi¹, Ariane Proteau¹, Magda Villarroya², Ismaïl Moukadiri², Linhua Zhang³, Jean-François Trempe¹, Allan Matte³, M. Eugenia Armengod², Miroslaw Cygler^1,3*

1 Department of Biochemistry, McGill University, Montréal, Québec, Canada, 2 Laboratorio de Genética Molecular, Centro de Investigación Príncipe Felipe, Valencia, Spain, 3 Biotechnology Research Institute, Montréal, Québec, Canada

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Highlight: "Our data support the role of frataxin as an iron donor for IscU to form the Fe-S clusters."

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A New Proposal For Achieving Neuronal Regeneration

Medical News Today, Article Date: 20 Apr 2010 - 2:00 PDT
Over the past four years, in the installations of the Faculty of Medicine, University of Cadiz, a group of researchers, headed by Doctor Carmen Estrada, has been studying neurogenesis the process of forming new neurons from mother cells in the adult mammal brain. The purpose of this research project is to contribute to the knowledge of this process with the object of finding some additional mechanism that would facilitate the treatment of pathologies of the central nervous system (CNS) that are suffered in consequence of neuronal death...

Frataxin, the mitochondrial iron chaperone for Fe-S cluster bioassembly

ProQuest, Dissertations & Theses

Jeremy David, Ph.D.,Wayne State University, 2010, 256 pages; AAT 3390960

Keywords: Iron, human disorders and diseases, Freidreich's Ataxia (FRDA),

frataxin, mitochondria, iron homeostasis, chaperone, Iron Sulfur Cluster (ISC),   ferrous iron, carboxylate ligands

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Erhaltene Reflexe, Propriozeption, SNAPs: trotzdem Friedreich-Ataxie - [Retained reflexes, proprioception, SNAPs: still Friedreich's ataxia]

Nervenarzt. 2010 Apr;81(4):442-3.
DOI: 10.1007/s00115-010-2946-3

 K. Dimitriadis^{1, 2}, S. Heck², M. Schubert¹ und T. Klopstock^{1, 2} 

(1)	Friedrich-Baur-Institut an der Neurologischen Klinik und Poliklinik, Klinikum der Universität München – Innenstadt, Ziemssenstraße 1a, 80336 München

(2)	Neurologische Klinik und Poliklinik, Klinikum der Universität München – Großhadern, München

 

Article in German

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Repligen Corporation - HDAC-3 Inhibitors for Friedreich's Ataxia

WALTHAM, Mass., April 16, 2010 /PRNewswire via COMTEX/ -- Repligen Corporation

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HDAC-3 Inhibitors for Friedreich's Ataxia
We are currently developing inhibitors of histone deacetylase 3 (HDAC-3) for the treatment of inherited neurodegenerative diseases such as Friedreich's ataxia.  Preclinical studies have shown that specific HDAC-3 inhibitors increase production of the protein frataxin which may have the potential to arrest disease progression in patients with Friedreich's ataxia.  We plan to file an Investigational New Drug Application (IND) for a Phase 1 human clinical study of RG2833 in healthy volunteers this quarter.

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Intermediate-Dose Idebenone and Quality of Life in Friedreich Ataxia

Pediatric Neurology
Volume 42, Issue 5, May 2010, Pages 338-342

n F. Brandsema MD^*, Derek Stephens MSc^†, Jessica Hartley MSc^‡ and Grace Yoon MD^{*, ‡}

^† Department of Child Health Evaluative Sciences, The Hospital for Sick Children, University of Toronto, Toronto, Canada
^‡ Division of Clinical and Metabolic Genetics, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada
^* Division of Neurology, The Hospital for Sick Children, University of Toronto, Toronto, Canada

Keywords: Idebenone, Friedreich ataxia, neurologic function, cardiac function, quality of life, 20 mg/kg per day, Pediatric Quality of Life Inventory,  International Cooperative Ataxia Rating Scale,  Activities of Daily Living Scale.

Health-Related Quality of Life in Children With Friedreich Ataxia

Pediatric Neurology
Volume 42, Issue 5, May 2010, Pages 335-337

Erin K. Paulsen BA^a, Lisa S. Friedman BA^a, Lauren M. Myers BA^a and David R. Lynch MD, PhD^{, a,}

^a Departments of Neurology and Pediatrics, University of Pennsylvania School of Medicine, and The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Keywords:  health-related quality of life scales,  Friedreich ataxia, children,  PedsQL 4.0, Multidimensional Fatigue Scale.

Iron redistribution as a therapeutic strategy for treating diseases of localized iron accumulation.

Can J Physiol Pharmacol. 2010 Mar;88(3):187-96.

Kakhlon O, Breuer W, Munnich A, Cabantchik ZI.

Department of Biological Chemistry, Alexander Silberman Institute of Life Sciences, The Hebrew University of Jerusalem, Safra Campus at Givat Ram, Jerusalem 91904, Israel.

Keywords:  Iron,  mitochondria, neurodegeneration, frataxin, Friedreich's ataxia (FRDA), iron chelation, deferiprone (DFP), iron-relocating abilities,  cellular iron misdistribution.

Efficacy of Riluzole in Hereditary Cerebellar Ataxia - This study is currently recruiting participants.

www.clinicaltrials.gov

This study is currently recruiting participants.

Verified by S. Andrea Hospital, April 2010

First Received: April 7, 2010   Last Updated: April 14, 2010   

Sponsor:	S. Andrea Hospital
Information provided by:	S. Andrea Hospital
ClinicalTrials.gov Identifier:	NCT01104649

Coenzyme Q10-responsive ataxia: 2-Year-treatment follow-up

Movement Disorders,  Volume 9999, Issue 9999 , PagesNA -(Articles online in advance of print)
DOI. 10.1002/mds.23129


Merce Pineda, MD, PhD ^{1 2}, Raquel Montero, PhD ^{2 3}, Asuncion Aracil, MD ^{1 2}, Mar M. O'Callaghan, MD ^{1 2}, Ana Mas, MD ⁴, Carmen Espinos, PhD ², Dolores Martinez-Rubio, BS ^{2 5}, Francesc Palau, MD, PhD ^{2 5}, Placido Navas, PhD ^{2 6}, Paz Briones, PhD ^{2 7}, Rafael Artuch, MD, PhD ^{2 3 *1}Department of Pediatric Neurology, Hospital Sant Joan de Déu, Barcelona, Spain
²Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), ISCIII, Spain
³Department of Clinical Biochemistry, Hospital Sant Joan de Déu, Barcelona, Spain
⁴Department of Pharmacy, Hospital Sant Joan de Déu, Barcelona, Spain
⁵Genetics and Medicine Molecular Unit, Instituto de Biomedicina de Valencia-CSIC, Valencia, Spain
⁶Centro Andaluz de Biología del Desarrollo, Universidad Pablo de Olavide, Sevilla, Spain
⁷Institut de Bioquímica Clínica, Hospital Clinic and CSIC, Barcelona, Spain

email: Rafael Artuch (rartuch@hsjdbcn.org)^*Correspondence to Rafael Artuch, Department of Clinical Biochemistry, Hospital Sant Joan de Déu, Passeig Sant Joan de Déu, 2, 08950 Esplugues, Barcelona, Spain

KEYWORDS: coenzyme Q₁₀ deficiency • mitochondrial disorders • ataxia • cerebellum • pediatric patients