Effects of cytoprotective antioxidants on lymphocytes from representative mitochondrial neurodegenerative diseases

Effects of cytoprotective antioxidants on lymphocytes from representative mitochondrial neurodegenerative diseases. Ruth Goldschmidt, Pablo M. Arce, Omar M. Khdour, Valérie C. Collin, Sriloy Dey, Jennifer Jaruvangsanti, David M. Fash, Sidney M. Hecht; Bioorganic & Medicinal Chemistry, Available online 12 December 2012. http://dx.doi.org/10.1016/j.bmc.2012.11.051.

Keywords: Idebenone, Friedreich's Ataxia, Adenosine triphosphate, Mitochondrial dysfunction, Antioxidants, Reactive oxygen species, Electron transport chain.

Expanded complexity of unstable repeat diseases

Expanded complexity of unstable repeat diseases. Urszula Polak, Elizabeth McIvor, Sharon Y.R. Dent, Robert D. Wells and Marek Napierala. Article first published online: 11 DEC 2012 | DOI: 10.1002/biof.1060

Keywords: Unstable repeat diseases (URDs), neurological diseases, repeat instability.

Leading international researchers awarded fellowships in UK

Leading international researchers awarded fellowships in UK 6 Dec 2012

The fifth round of Newton International Fellowships has been awarded jointly by the UK’s national research academies – the British Academy and the Royal Society. Fellowships have been awarded to 41 successful applicants who be investigating topics ranging from the socio-economic impact on women in Ghanaian oil communities to boosting visual short-term memory. They come from research institutes in over 20 different countries including Thailand, Mexico, China and Australia.

Dr Matija Popovic, Croatia - Understanding the molecular basis of frataxin turn-over – hosted by National Institute for Medical Research

Transcriptional Regulation by Nuclear Corepressors and PGC-1α: Implications for Mitochondrial Quality Control and Insulin Sensitivity

Transcriptional Regulation by Nuclear Corepressors and PGC-1α: Implications for Mitochondrial Quality Control and Insulin Sensitivity; Zhengtang Qi and Shuzhe Ding, PPAR Research Volume 2012 (2012), Article ID 348245, 12 pages doi:10.1155/2012/348245

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Involvement of PGC-1α in the formation and maintenance of neuronal dendritic spines

Involvement of PGC-1α in the formation and maintenance of neuronal dendritic spines. Aiwu Cheng, Ruiqian Wan, Jenq-Lin Yang, Naomi Kamimura, Tae Gen Son, Xin Ouyang, Yongquan Luo, Eitan Okun & Mark P. Mattson. Nature Communications 3, Article number: 1250; doi:10.1038/ncomms2238

Keywords: synapses, neuronal circuits, peroxisome proliferator-activated receptor γ co-activator 1α, mitochondrial biogenesis, dendritic spines, hippocampal neurons, dendritic spines.

Dr Tremblay: Development of a potential therapy for Friedreich ataxia

Dr Tremblay: Development of a potential therapy for Friedreich ataxia (english)
04/12/2012 by Acaf Fondation Claude St-Jean

Iron binding activity is essential for the function of IscA in iron-sulphur cluster biogenesis

Iron binding activity is essential for the function of IscA in iron-sulphur cluster biogenesis; Aaron P. Landry , Zishuo Cheng and Huangen Ding; Dalton Trans., 2012, Accepted Manuscript. DOI: 10.1039/C2DT32000B

Keywords: Iron-sulphide cluster biogenesis, iron, sulphide, scaffold proteins, L-cysteine, cysteine desulfurases, IscA.

Clinical use of frataxin measurement in a patient with a novel deletion in the FXN gene.

Clinical use of frataxin measurement in a patient with a novel deletion in the FXN gene.Saccà F, Marsili A, Puorro G, Antenora A, Pane C, Tessa A, Scoppettuolo P, Nesti C, Brescia Morra V, De Michele G, Santorelli FM, Filla A; Journal of neurology : 2012 Nov 30

Keywords: Friedreich ataxia (FRDA), FXN gene, frataxin, point mutation, novel deletion in exon 5a (c.572delC).

Calorie Restriction and SIRT3 Trigger Global Reprogramming of the Mitochondrial Protein Acetylome

Calorie Restriction and SIRT3 Trigger Global Reprogramming of the Mitochondrial Protein Acetylome Alexander S. Hebert, Kristin E. Dittenhafer-Reed, Wei Yu, Derek J. Bailey, Ebru Selin Selen, Melissa D. Boersma, Joshua J. Carson, Marco Tonelli, Allison J. Balloon, Alan J. Higbee, Michael S. Westphall, David J. Pagliarini, Tomas A. Prolla, Fariba Assadi-Porter, Sushmita Roy, John M. Denu, Joshua J. Coon;Molecular Cell, Available online 29 November 2012

The author explain that frataxin, and other proteins involved in iron homeostasis are well-represented as major targets of SIRT3 deacetylation, and even say the level and the exact place the interaction in the protein.

Synthesis and Biological Activities of N-(3-Carboxylpropyl)-5-amino-2-hydroxy-3-tridecyl-1,4-benzoquinone and Analogues.

Synthesis and Biological Activities of N-(3-Carboxylpropyl)-5-amino-2-hydroxy-3-tridecyl-1,4-benzoquinone and Analogues.Madathil MM, Khdour OM, Jaruvangsanti J, Hecht SM.; J Nat Prod. 2012 Nov 28. [Epub ahead of print]

KEYWORDS: benzoquinone, natural product, Friedreich's ataxia (FRDA) lymphocytes, oxidative stress, cytoprotection.