Antioxidant targeting by deferiprone in diseases related to oxidative damage

Antioxidant targeting by deferiprone in diseases related to oxidative damage . Kontoghiorghe CN, Kolnagou A, Kontoghiorghes GJ.; Front Biosci (Landmark Ed). 2014 Jun 1;19:862-885. http://dx.doi.org/10.2741/4253

Keywords:antioxidant pharmaceuticals, free radical damage (FRD), Deferiprone (L1), iron overload, cardiomyopathy, acute kidney disease, Friedreich ataxia.

Myelin paucity of the superior cerebellar peduncle in individuals with Friedreich ataxia: an MRI magnetization transfer imaging study

Myelin paucity of the superior cerebellar peduncle in individuals with Friedreich ataxia: an MRI magnetization transfer imaging study. Louise A. Corben, Saman Rassaei Kashuk, Hamed Akhlaghi, Sharna Jamadar, Martin B. Delatycki, Joanne Fielding, Beth Johnson, Nellie Georgiou-Karistianis, Gary F. Egan, Journal of the Neurological Sciences, Available online 1 June 2014, ISSN 0022-510X, http://dx.doi.org/10.1016/j.jns.2014.05.057.

This is the first study to demonstrate reduced MTR in the SCP of individuals with FRDA when compared to control participants. These findings may reflect a process of disruption of the reciprocal relationship between oligodendrocytes and axons resulting in paucity of myelin, and provides significant new insight into the neurodegenerative pathology of FRDA

Photoinduced Transformation of Iron Chelator Deferiprone: Possible Implications in Drug Metabolism and Toxicity

Photoinduced Transformation of Iron Chelator Deferiprone: Possible Implications in Drug Metabolism and Toxicity. V.A. Timoshnikov, V.I. Klimentiev, N.E. Polyakov, G.J. Kontoghiorghes, Journal of Photochemistry and Photobiology A: Chemistry, Available online 2 June 2014, ISSN 1010-6030, http://dx.doi.org/10.1016/j.jphotochem.2014.05.012.

Thefindings suggest that high dose of sunlight might cause chemical modifications which may have chemical, biochemical, pharmacological and toxicological implications, especially possible undesirable phototoxic effects in patients undergoing treatment in countries with increased sunlight exposure.

C9orf72; abnormal RNA expression is the key

C9orf72; abnormal RNA expression is the key. Peter Heutink, Iris E. Jansen, Emily M. Lynes, Experimental Neurology, Available online 27 May 2014, ISSN 0014-4886, http://dx.doi.org/10.1016/j.expneurol.2014.05.020.

Despite differences with Friedreich's ataxia, hexanucleotide repeat instead of trinucleotide repeat...., it could be many points in common with the FA, as the loss of protein expression and toxic RNA species. It is interesting the authors's hypothesis about the mechanisms leading to pathology.

Low Bone Mineral Density in Friedreich Ataxia.

Low Bone Mineral Density in Friedreich Ataxia. Eigentler A1, Nachbauer W, Donnemiller E, Poewe W, Gasser RW, Boesch S.; Cerebellum. 2014 May 25. [Epub ahead of print]

Managing Dysphagia Through Diet Modifications

Managing Dysphagia Through Diet Modifications. Garcia, Jane Mertz PhD, CCC-SLP; Chambers, Edgar IV PhD; AJN, American Journal of Nursing: November 2010 - Volume 110 - Issue 11 - pp 26-33; doi: 10.1097/01.NAJ.0000390519.83887.02

Keywords: dysphagia, impaired swallowing, dietary modification, malnutrition, aspiration pneumonia

Tissue specificity in DNA repair: lessons from trinucleotide repeat instability

Tissue specificity in DNA repair: lessons from trinucleotide repeat instability. Vincent Dion; Trends in Genetics, Available online 16 May 2014, ISSN 0168-9525, http://dx.doi.org/10.1016/j.tig.2014.04.005.

Keywords: DNA repair; genome stability; trinucleotide repeat instability; base excision repair; single-strand break repair; nucleotide excision repair; tissue-specific DNA repair

Gene therapy protects heart in Friedreich's ataxia

Gene therapy protects heart in Friedreich's ataxia. Alexandra Flemming; Nature Reviews Drug Discovery (2014); doi:10.1038/nrd4346; Published online 23 May 2014

Herzbeteiligung bei Friedreich-Ataxie / Heart involvement in Friedreich's ataxia.

Herzbeteiligung bei Friedreich-Ataxie / Heart involvement in Friedreich's ataxia. Weidemann F, Scholz F, Florescu C, Liu D, Hu K, Herrmann S, Ertl G, Störk S.; Herz. 2014 May 23. DOI 10.1007/s00059-014-4097-y

[Article in German]

Keywords: Mitochondriale Funktion, Pathophysiologie, Frataxin, Kardiomyopathie, Herzinsuffizienz, Mitochondrial function, Pathophysiology, Frataxin, Cardiomyopathy, Heart failure

Spécificités de la prise en charge anesthésique chez les patients souffrant de maladie neurologique : éclairage sur l’anesthésie locorégionale/Anaesthetic management of patient with neurological disease: Focus on regional anaesthesia

Spécificités de la prise en charge anesthésique chez les patients souffrant de maladie neurologique : éclairage sur l’anesthésie locorégionale; Jean-Marc Malinovsky, Armine Hamidi, Chantal Lelarge, Catherine Boulay-Malinovsky, La Presse Médicale, Available online 20 May 2014, ISSN 0755-4982, http://dx.doi.org/10.1016/j.lpm.2013.11.028.

Anaesthetic management of patient with neurological disease: Focus on regional anaesthesia

Maladie de Friedreich: Les anesthésies locorégionales ne sont pas contre-indiquées et l’efficacité des anesthésies périmédullaires a déjà été rapportée.