Instituto de Biomedicina de Valencia -ko ikertzaileekin lankidetzan egindako argitalpenak (13)

2020

  1. Cofilin dysregulation alters actin turnover in frataxin-deficient neurons

    Scientific Reports, Vol. 10, Núm. 1

2019

  1. Phosphodiesterase Inhibitors Revert Axonal Dystrophy in Friedreich’s Ataxia Mouse Model

    Neurotherapeutics, Vol. 16, Núm. 2, pp. 432-449

2017

  1. Reversible axonal dystrophy by calcium modulation in frataxin-deficient sensory neurons of YG8R mice

    Frontiers in Molecular Neuroscience, Vol. 10

2014

  1. Mitochondrial dysfunction induced by frataxin deficiency is associated with cellular senescence and abnormal calcium metabolism

    Frontiers in Cellular Neuroscience, Vol. 8, Núm. MAY

2013

  1. Mitochondrial pathophysiology in Friedreich's ataxia

    Journal of Neurochemistry, Vol. 126, Núm. SUPPL.1, pp. 53-64

2011

  1. Differential expression of PGC-1α and metabolic sensors suggest age-dependent induction of mitochondrial biogenesis in friedreich ataxia fibroblasts

    PLoS ONE, Vol. 6, Núm. 6

  2. Disruption of the ATP-binding cassette B7 (ABTM-1/ABCB7) induces oxidative stress and premature cell death in Caenorhabditis elegans

    Journal of Biological Chemistry, Vol. 286, Núm. 24, pp. 21304-21314

2010

  1. Flavin adenine dinucleotide rescues the phenotype of frataxin deficiency

    PLoS ONE, Vol. 5, Núm. 1

2009

  1. Friedreich ataxia: An update on animal models, frataxin function and therapies

    Advances in Experimental Medicine and Biology

2007

  1. The frataxin-encoding operon of Caenorhabditis elegans shows complex structure and regulation

    Genomics, Vol. 89, Núm. 3, pp. 392-401

2006

  1. Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant

    FASEB Journal, Vol. 20, Núm. 1, pp. 172-174

2005

  1. Autosomal recessive cerebellar ataxias. Their classification, genetic features and pathophysiology

    Revista de Neurologia, Vol. 41, Núm. 7, pp. 409-422

  2. Frataxin interacts functionally with mitochondrial electron transport chain proteins

    Human Molecular Genetics, Vol. 14, Núm. 15, pp. 2091-2098