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   <ow:Publication rdf:about="oai:digibug.ugr.es:10481/100992">
      <dc:title>Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model</dc:title>
      <dc:creator>Ranea-Robles, Pablo</dc:creator>
      <dc:subject>RIP140</dc:subject>
      <dc:subject>mitochondria</dc:subject>
      <dc:subject>oxidative stress</dc:subject>
      <dc:subject>peroxisome</dc:subject>
      <dc:description>Se sube versión original del autor, tal y como está en el repositorio de la UPF (http://hdl.handle.net/10230/53662)</dc:description>
      <dc:description>Aims: Mitochondrial dysfunction and inflammation are at the core of axonal degeneration in several multifactorial neurodegenerative diseases, including multiple sclerosis, Alzheimer's disease, and Parkinson's disease. The transcriptional coregulator RIP140/NRIP1 (receptor-interacting protein 140) modulates these functions in liver and adipose tissue, but its role in the nervous system remains unexplored. Here, we investigated the impact of RIP140 in the Abcd1- mouse model of X-linked adrenoleukodystrophy (X-ALD), a genetic model of chronic axonopathy involving the convergence of redox imbalance, bioenergetic failure, and chronic inflammation.&#xd;
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Methods and results: We provide evidence that RIP140 is modulated through a redox-dependent mechanism driven by very long-chain fatty acids (VLCFAs), the levels of which are increased in X-ALD. Genetic inactivation of RIP140 prevented mitochondrial depletion and dysfunction, bioenergetic failure, inflammatory dysregulation, axonal degeneration and associated locomotor disabilities in vivo in X-ALD mouse models.&#xd;
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Conclusions: Together, these findings show that aberrant overactivation of RIP140 promotes neurodegeneration in X-ALD, underscoring its potential as a therapeutic target for X-ALD and other neurodegenerative disorders that present with metabolic and inflammatory dyshomeostasis.</dc:description>
      <dc:date>2025-01-29T13:05:52Z</dc:date>
      <dc:date>2025-01-29T13:05:52Z</dc:date>
      <dc:date>2021-07-29</dc:date>
      <dc:type>journal article</dc:type>
      <dc:identifier>Ranea-Robles P, Galino J, Espinosa L, Schlüter A, Ruiz M, Calingasan NY, Villarroya F, Naudí A, Pamplona R, Ferrer I, Beal MF, Portero-Otín M, Fourcade S and Pujol A. Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model. Neuropathol Appl Neurobiol. 2022 Feb; 48(1):e12747 doi: 10.1111/nan.12747. Epub 2021 Jul 29.</dc:identifier>
      <dc:identifier>https://hdl.handle.net/10481/100992</dc:identifier>
      <dc:identifier>10.1111/nan.12747</dc:identifier>
      <dc:language>eng</dc:language>
      <dc:rights>http://creativecommons.org/licenses/by-nc-nd/4.0/</dc:rights>
      <dc:rights>open access</dc:rights>
      <dc:rights>Attribution-NonCommercial-NoDerivatives 4.0 Internacional</dc:rights>
   </ow:Publication>
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