Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model,Neuropathology and Applied Neurobiology

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Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model
Neuropathology and Applied Neurobiology ( IF 4.0 ) Pub Date : 2021-07-08 , DOI: 10.1111/nan.12747
Pablo Ranea-Robles _{1,

2,

3} , Jorge Galino _{1,

2} , Lluís Espinosa ₄ , Agatha Schlüter _{1,

2} , Montserrat Ruiz _{1,

2} , Noel Ylagan Calingasan ₅ , Francesc Villarroya _{6,

7} , Alba Naudí ₈ , Reinald Pamplona ₈ , Isidre Ferrer _{9,

10,

11,

12} , M Flint Beal ₅ , Manuel Portero-Otín ₈ , Stéphane Fourcade _{1,

2} , Aurora Pujol _{1,

2,

13}

Affiliation

Neurometabolic Diseases Laboratory, Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain.
CIBERER U759, Center for Biomedical Research on Rare Diseases, ISCIII, Madrid, Spain.
Department of Genetics and Genomic Sciences, Icahn Institute for Data Science and Genomic Technology, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
Institut Municipal d'Investigacions Mèdiques, Hospital del Mar, Barcelona, Spain.
Feil Family Brain and Mind Research Institute, Weill Cornell Medical College, New York, New York, USA.
Department of Biochemistry and Molecular Biomedicine and Institut de Biomedicina, University of Barcelona, Barcelona, Catalonia, Spain.
Fisiopatología de la Obesidad y Nutrición, CIBER, Madrid, Spain.
Experimental Medicine Department, University of Lleida-IRBLleida, Lleida, Spain.
Department of Pathology and Experimental Therapeutics, University of Barcelona, Barcelona, Spain.
Center for Biomedical Research on Neurodegenerative Diseases (CIBERNED), ISCIII, Madrid, Spain.
Institute of Neurosciences, University of Barcelona, Barcelona, Spain.
Neuropathology, Bellvitge University Hospital-Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain.
Catalan Institution of Research and Advanced Studies (ICREA), Barcelona, Spain.

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.

中文翻译：

通过 RIP140 调节线粒体和炎症稳态在肾上腺脑白质营养不良小鼠模型中具有神经保护作用

线粒体功能障碍和炎症是几种多因素神经退行性疾病（包括多发性硬化症、阿尔茨海默病和帕金森病）中轴突变性的核心。转录共调节因子 RIP140/NRIP1（受体相互作用蛋白 140）在肝脏和脂肪组织中调节这些功能，但其在神经系统中的作用仍未得到探索。在这里，我们研究了 RIP140 在Abcd1 ^- X 连锁肾上腺脑白质营养不良 (X-ALD) 小鼠模型中的影响，这是一种慢性轴索病的遗传模型，涉及氧化还原失衡、生物能量衰竭和慢性炎症的收敛。

更新日期：2021-07-08

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