Thursday, October 21, 2010

Frataxin is an important regulator of iron metabolism

MRC, National Institute for Medical Research, 20 October 2010

Brief summary of the article by Dr. Annalisa Pastore, explaining their research in words more simple and comprehensible to all

Frataxin depletion in yeast triggers upregulation of iron transport systems before affecting iron-sulfur enzyme activities.

J Biol Chem. 2010 Oct 18. [Epub ahead of print]
Moreno-Cermeno A, Obis E, Belli G, Cabiscol E, Ros J, Tamarit J.

University of Lleida, Spain.

Keywords: frataxin, a mitochondrial protein, iron homeostasis, yeast model, frataxin homolog YFH1, aconitase, secondary events, iron overloading, superoxide dismutase, protein carbonyl formation, manganese, metal ion transporter Smf2.

FULL TEXT PDF

Wednesday, October 20, 2010

Pharmacokinetic evaluation of idebenone.

Expert Opin Drug Metab Toxicol. 2010 Nov;6(11):1437-44.

Becker C, Bray-French K, Drewe J.
Basel Pharmacoepidemiology Unit, University Hospital Basel, Switzerland.

Conclusions: Idebenone shows dose-proportional pharmacokinetics in healthy subjects in daily doses up to 2250 mg, and was safe and well tolerated at doses up to 2250 mg/day, in clinical studies.

Pathogenesis of impaired glucose tolerance and diabetes in Friedreich's ataxia: contribution of insulin resistance and pancreatic beta cell dysfunction.

The free library,
Miriam Cnop, MD, PhD, Universite Libre de Bruxelles, Brussels, Belgium

Research summary

Keywords: pancreatic b cells, Friedreich's ataxia, insulin demand.

Tuesday, October 19, 2010

Structural bases for the interaction of frataxin with the central components of iron–sulphur cluster assembly

Nature Communications, Volume:1, Article number: 95, DOI: doi:10.1038/ncomms1097
Received 08 June 2010, Accepted 22 September 2010, Published 19 October 2010

National Institute for Medical Research, The Ridgeway, London NW7 1AA, UK.
* Filippo Prischi,
* Clara Iannuzzi,
* Chiara Pastore,
* Salvatore Adinolfi,
* Stephen R. Martin &
* Annalisa Pastore
European Molecular Biology Laboratory, EMBL c/o DESY, Notkestrasse 85, Hamburg D-22603, Germany.
* Petr V. Konarev &
* Dmitri I. Svergun

OPEN ARTICLE

Mitochondrial ROS Generation and Its Regulation: Mechanisms Involved in H2O2 Signaling

Antioxidants & Redox Signaling. Online Ahead of Print: October 18, 2010, doi:10.1089/ars.2010.3363.
Michel Rigoulet, Edgar D. Yoboue, Anne Devin. Université Bordeaux 2, and Institute of Biochemistry and Genetics of the Cell (IBGC) du CNRS, Bordeaux, France.

Keyworsd: Mitochondria, reactive oxygen species, oxidative stress, respiratory chain, dehydrogenases, bc1 complex, complex I, signaling molecules.

Public summary of opinion on orphan designationN-(6-(2-aminophenylamino)-6-oxohexyl)-4-methylbenzamide for the treatment of Friedreich’s ataxia,

12 October 2010
EMA/COMP/455242/2010
Committee for Orphan Medicinal Products.

On 1 October 2010, orphan designation (EU/3/10/793) was granted by the European Commission to Repligen Europe Limited, Ireland, for N-(6-(2-aminophenylamino)-6-oxohexyl)-4-methylbenzamide for the treatment of Friedreich’s ataxia.

Sunday, October 17, 2010

Induced pluripotency: history, mechanisms, and applications

Genes & Dev. 2010. 24: 2239-2263, doi: 10.1101/gad.1963910
Matthias Stadtfeld, Konrad Hochedlinger

(REVIEWS)

OPEN ACCESS

The generation of induced pluripotent stem cells (iPSCs) from somatic cells demonstrated that adult mammalian cells can be reprogrammed to a pluripotent state by the enforced expression of a few embryonic transcription factors.

FULL TEXT PDF

Thursday, October 14, 2010

In vivo evidence for the iron binding activity of an iron-sulfur cluster assembly protein IscA in Escherichia co

Biochem. J. (2010) Immediate Publication, doi:10.1042/BJ20101507

Wu Wang, Hao Huang, Guoqiang Tan, Fan Si, Min Liu, Aaron P. Landry, Jianxin Lu and Huangen Ding
Biological Sciences, Louisiana State University, Baton Rouge, LA 70803, U.S.A.

"IscU, a proposed iron-sulfur cluster assembly scaffold protein, and CyaY, a bacterial frataxin homolog that has been postulated as an iron donor for the iron-sulfur cluster assembly, fail to bind any iron in E. coli cells under the same experimental conditions"

FULL TEXT PDF

Epigenetic modifications and human disease

Nature Biotechnology, Volume:28, Pages:1057–1068,Year published:(2010)
DOI:10.1038/nbt.1685, Published online 13 October 2010

Anna Portela & Manel Esteller
Cancer Epigenetics and Biology Program (PEBC), Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Catalonia, Spain.
Manel Esteller
Institucio Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Catalonia, Spain.

Epigenetic modifications in neurodegenerative and neurological diseases (Friedreich's ataxia) , Epigenetic modifications in neurodevelopmental disorder

FULL TEXT