Mitochondria in Pediatric Cardiovascular Diseases

Mitochondria in Pediatric Cardiovascular Diseases. José Marín-García M.D.,
Mitochondria and Their Role in Cardiovascular Disease, 2013, pp 245-270

Keywords: cardiac mitochondrial respiratory enzymes, dilated and hypertrophic cardiomyopathy, mitochondrial cardiomyopathy, neurological disorders, cardioneuropathies.

The molecular biology of Friedreich ataxia

JBC Podcasts: The molecular biology of Friedreich ataxia.

Interview of ELENA HIDALGO, Principal Investigator, Department of Experimental and Health Sciences, Universitat Pompeu Fabra, Barcelona, Spain

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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.