Substantia Nigra Echogenicity in Friedreich’s Ataxia Patients

Substantia Nigra Echogenicity in Friedreich’s Ataxia Patients. María Sierra, Jon Infante,
José Berciano. The Cerebellum, December 2012.

Keeywords: substantia nigra (SN) hypoechogenicity, Friedreich’s ataxia (FRDA), restless syndrome (RLS).

[Late-onset Friedreich's ataxia with preservation of osteomuscular reflexes].

[Late-onset Friedreich's ataxia with preservation of osteomuscular reflexes]. Garcia-Estevez DA. Rev Neurol. 2012 Dec 16;55(12):765-7.

[Article in Spanish]

Autophagy induction extends lifespan and reduces lipids content in response to frataxin silencing in C. elegans

Autophagy induction extends lifespan and reduces lipids content in response to frataxin silencing in C. elegans.. Alfonso Schiavi, Alessandro Torgovnick, Alison Kell, Evgenia Megalou, Natascha Castelein, Ilaria Guccini, Laura Marzocchella, Sara Gelino, Malene Hansen, Florence Malisan, Ivano Condo’, Roberto Bei, Shane Rea, Bart P. Braeckman, Nektarios Tavernarakis, Roberto Testi, Natascia Ventura. Experimental Gerontology, Available online 13 December 2012; http://dx.doi.org/10.1016/j.exger.2012.12.002,

Keywords: aging, nematode, mitochondria, frataxin, p53/cep-1, autophagy, fat, lipid metabolism, Beclin, Friedreich ataxia.

Common data elements for clinical research in Friedreich's ataxia

Common data elements for clinical research in Friedreich's ataxia. David R. Lynch MD PhD, Massimo Pandolfo MD, Jorg B. Schulz MD, Susan Perlman MD, Martin B. Delatycki MD PhD, R. Mark Payne MD, Robert Shaddy MD, Kenneth H. Fischbeck MD, Jennifer Farmer MS, Paul Kantor MD, Subha V. Raman MD, Lisa Hunegs MSW MPH, Joanne Odenkirchen MPH, Kristy Miller MPH, Petra Kaufmann MD MSC. Movement Disorders; Article first published online: 12 DEC 2012 | DOI: 10.1002/mds.25201.

Keywords: cerebellum, dorsal root ganglion, mitochondrion, clinical measure, NINDS Common Data Elements.

Fifth Annual Friedreich’s Ataxia Symposium at The Children's Hospital of Philadelphia

The Fifth Annual Friedreich’s Ataxia Symposium was held on Oct. 8, 2012

Topics and presentations from previous Friedreich's Ataxia symposia.

Proyecto: "Cerebelo y Cognición: deterioro neuropsicológico en la Ataxia de Friedreich y su relación con la degeneración cerebelosa en imágenes de RM".

"Cerebelo y Cognición: deterioro neuropsicológico en la Ataxia de Friedreich y su relación con la degeneración cerebelosa en imágenes de RM".

NUEVA CONVOCATORIA DE LA UNIVERSIDAD DE LA LAGUNA PARA LA CONTRATACIÓN DE PERSONAL LABORAL TEMPORAL, MEDIANTE EL SISTEMA DE CONCURSO PÚBLICO, CON CARGO A VARIOS PROYECTOS DE INVESTIGACIÓN (CONVOCATORIA PUBLICADA EN B.O.C. Nº 240, DE 10 DE DICIEMBRE D 11/12/2012

Proyecto 2 (nº referencia: 2012_83):

Título del proyecto: "Cerebelo y Cognición: deterioro neuropsicológico en la Ataxia de Friedreich y su relación con la degeneración cerebelosa en imágenes de RM".

Departamento de destino: Dpto. de Psicobiología y Metodología de las Ciencias del Comportamiento. (Facultad de Psicología).

Nº de plazas: 1 plaza

Titulación académica requerida: Licenciado/a en Psicología.

Duración del contrato: 9 meses (prorrogable según disponibilidad presupuestaria).

Extranuclear Localization of SIRT1 and PGC-1α: An Insight into Possible Roles in Diseases Associated with Mitochondrial Dysfunction

Extranuclear Localization of SIRT1 and PGC-1α: An Insight into Possible Roles in Diseases Associated with Mitochondrial Dysfunction . Aquilano, K.; Baldelli, S.; Pagliei, B.; R. Ciriolo, M.; Current Molecular Medicine, Volume 13, Number 1, January 2013 , pp. 140-154(15). DOI: http://dx.doi.org/10.2174/156652413804486241

Keywords: Biogenesis, cellular metabolism, cytoplasm, mitochondria, mitochondrial diseases, transcriptional regulation, SIRT1, PGC-1α, neurodegenerative disorders, myopathies,Type II diabetes mellitus.

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