Browsing Articles publicats (IRBLleida) by Author "Delaspre, Fabien"
Now showing items 1-4 of 4
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Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment
Britti, Elena; Delaspre, Fabien; Feldman, Anat; Osborne, Melissa; Greif, Hagar; Tamarit Sumalla, Jordi; Ros Salvador, Joaquim (Wiley Open Access, 2018)Friedreich ataxia (FA) is a rare disease caused by deficiency of frataxin, a mitochondrial protein. As there is no cure available for this disease, many strategies have been developed to reduce the deleterious effects of ... -
Mice harboring the FXN I151F pathological point mutation present decreased frataxin levels, a Friedreich ataxia-like phenotype, and mitochondrial alterations
Medina Carbonero, Marta; Sanz Alcázar, Arabela; Britti, Elena; Delaspre, Fabien; Cabiscol Català, Elisa; Ros Salvador, Joaquim; Tamarit Sumalla, Jordi (Springer; Birkhäuser Verlag, 2022)Friedreich Ataxia (FA) is a rare neuro-cardiodegenerative disease caused by mutations in the frataxin (FXN) gene. The most prevalent mutation is a GAA expansion in the first intron of the gene causing decreased frataxin ... -
Mitochondrial calcium signaling and neurodegenerative diseases
Britti, Elena; Delaspre, Fabien; Tamarit Sumalla, Jordi; Ros Salvador, Joaquim (Portland Press, 2018-11-16)Calcium is utilised by cells in signalling and in regulating ATP production; it also contributes to cell survival and, when concentrations are unbalanced, triggers pathways for cell death. Mitochondria contribute to calcium ... -
PPAR gamma agonist leriglitazone improves frataxin-loss impairments in cellular and animal models of Friedreich Ataxia
Rodríguez-Pascau, Laura; Britti, Elena; Calap Quintana, Pablo; Na Dong, Yi; Vergara, Cristina; Delaspre, Fabien; Medina Carbonero, Marta; Tamarit Sumalla, Jordi; Pallardó, Federico V.; Gonzalez Cabo, Pilar; Ros Salvador, Joaquim; Lynch, David R.; Martinell, Marc; Pizcueta, Pilar (Elsevier, 2021)Friedreich ataxia (FRDA), the most common autosomal recessive ataxia, is characterized by degeneration of the large sensory neurons and spinocerebellar tracts, cardiomyopathy, and increased incidence in diabetes. The ...