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.

The mitochondrial protein frataxin is downregulated in hemodialysis patients.

The mitochondrial protein frataxin is downregulated in hemodialysis patients.Hasuike Y, Nagai T, Yorifuji S, Tanaka S, Matsumoto A, Yahiro M, Kaibe S, Kida A, Tokuyama M, Nagasawa Y, Otaki Y, Kuragano T, Nakanishi T.; Clin Exp Nephrol. 2012 Nov 23. [Epub ahead of print]

Keywords: frataxin, iron metabolism, heme, iron sulfur cluster synthesis, oxidative stress, uremia, polymorphonuclear leukocytes (PMNLs), hemodialysis (HD), frataxin/glyceraldehyde-3-phosphate dehydrogenase mRNA ratio, malondialdehyde, cytokine tumor necrosis factor-α, frataxin expression.

Novel Therapeutic Challenges in Cerebellar Diseases

Novel Therapeutic Challenges in Cerebellar Diseases. Dr. Antoni Matilla-Dueñas, Dr. Carme Serrano, Dr. Yerko Ivánovic, Dr. Ramiro Alvarez, Dr. Pilar Latorre, Dr. David Genís. Handbook of the Cerebellum and Cerebellar Disorders, 2013, pp 2370-2394.

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Characterizing POLG Ataxia: Clinics, Electrophysiology and Imaging.

Characterizing POLG Ataxia: Clinics, Electrophysiology and Imaging. Synofzik M, Srulijes K, Godau J, Berg D, Schöls L; Cerebellum (London, England) [2012, 11(4):1002-1011]

Keywords: mitochondrial DNA polymerase gamma (POLG), POLG-associated ataxia (POLG-A), standardized clinical investigation, nerve conduction studies, motor-evoked potentials, magnetic resonance imaging (MRI), transcranial sonography (TCS), Friedreich's ataxia (FA), sensory axonal polyneuropathy type.