Neurobiology of rare diseases - Neurobiologia de malalties minoritàries (NEURADIS)
2021 SGR 00323
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Instituto de Investigación Biomédica de Lleida
Lleida, EspañaPublicaciones en colaboración con investigadores/as de Instituto de Investigación Biomédica de Lleida (57)
2024
2023
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NOD mouse dorsal root ganglia display morphological and gene expression defects before and during autoimmune diabetes development
Frontiers in Endocrinology, Vol. 14
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Oxidative stress-induced FAK activation contributes to uterine serous carcinoma aggressiveness
Molecular Oncology, Vol. 17, Núm. 1, pp. 98-118
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Persistent NRG1 Type III Overexpression in Spinal Motor Neurons Has No Therapeutic Effect on ALS-Related Pathology in SOD1G93A Mice
Neurotherapeutics, Vol. 20, Núm. 6, pp. 1820-1834
2022
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Accumulation of misfolded SOD1 outlines distinct patterns of motor neuron pathology and death during disease progression in a SOD1G93A mouse model of amyotrophic lateral sclerosis
Brain Pathology, Vol. 32, Núm. 6
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Evolution of an Iron-Detoxifying Protein: Eukaryotic and Rickettsia Frataxins Contain a Conserved Site Which Is Not Present in Their Bacterial Homologues
International Journal of Molecular Sciences, Vol. 23, Núm. 21
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Mice harboring the FXN I151F pathological point mutation present decreased frataxin levels, a Friedreich ataxia-like phenotype, and mitochondrial alterations
Cellular and Molecular Life Sciences, Vol. 79, Núm. 2
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SMN Is Physiologically Downregulated at Wild-Type Motor Nerve Terminals but Aggregates Together with Neurofilaments in SMA Mouse Models
Biomolecules, Vol. 12, Núm. 10
2021
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Calcitriol increases frataxin levels and restores mitochondrial function in cell models of Friedreich Ataxia
Biochemical Journal, Vol. 478, Núm. 1, pp. 1-20
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Calpain-Inhibitors Protect Frataxin-Deficient Dorsal Root Ganglia Neurons from Loss of Mitochondrial Na+/Ca2+ Exchanger, NCLX, and Apoptosis
Neurochemical Research, Vol. 46, Núm. 1, pp. 108-119
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Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1
Autophagy, Vol. 17, Núm. 1, pp. 1-382
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Microglial recruitment and mechanisms involved in the disruption of afferent synaptic terminals on spinal cord motor neurons after acute peripheral nerve injury
GLIA, Vol. 69, Núm. 5, pp. 1216-1240
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Mitochondrial iron and calcium homeostasis in Friedreich ataxia
IUBMB Life
2020
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Frataxin-deficient cardiomyocytes present an altered thiol-redox state which targets actin and pyruvate dehydrogenase
Redox Biology, Vol. 32
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Mitochondrial localization of the yeast forkhead factor hcm1
International Journal of Molecular Sciences, Vol. 21, Núm. 24, pp. 1-11
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Nusinersen ameliorates motor function and prevents motoneuron Cajal body disassembly and abnormal poly(A) RNA distribution in a SMA mouse model
Scientific Reports, Vol. 10, Núm. 1
2019
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Loss of glutathione redox homeostasis impairs proteostasis by inhibiting autophagy-dependent protein degradation
Cell Death and Differentiation, Vol. 26, Núm. 9, pp. 1545-1565
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Redox control of yeast Sir2 activity is involved in acetic acid resistance and longevity
Redox Biology, Vol. 24
2018
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Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA
Scientific Reports, Vol. 8, Núm. 1
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Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment
Journal of Cellular and Molecular Medicine, Vol. 22, Núm. 2, pp. 834-848