Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model

P Ranea‐Robles, J Galino, L Espinosa… - Neuropathology and …, 2022 - Wiley Online Library
Aims Mitochondrial dysfunction and inflammation are at the core of axonal degeneration in
several multifactorial neurodegenerative diseases, including multiple sclerosis, Alzheimer's …

Aberrant regulation of the GSK‐3β/NRF 2 axis unveils a novel therapy for adrenoleukodystrophy

P Ranea‐Robles, N Launay, M Ruiz… - EMBO molecular …, 2018 - embopress.org
The nuclear factor erythroid 2‐like 2 (NRF 2) is the master regulator of endogenous
antioxidant responses. Oxidative damage is a shared and early‐appearing feature in X …

[HTML][HTML] Oxidative stress, mitochondrial and proteostasis malfunction in adrenoleukodystrophy: a paradigm for axonal degeneration

S Fourcade, I Ferrer, A Pujol - Free Radical Biology and Medicine, 2015 - Elsevier
Peroxisomal and mitochondrial malfunction, which are highly intertwined through redox
regulation, in combination with defective proteostasis, are hallmarks of the most prevalent …

Imbalanced mitochondrial dynamics contributes to the pathogenesis of X-linked adrenoleukodystrophy

N Launay, J Lopez-Erauskin, P Bianchi, S Guha… - Brain, 2024 - academic.oup.com
The peroxisomal disease adrenoleukodystrophy (X-ALD) is caused by loss of the transporter
of very-long-chain fatty acids (VLCFAs), ABCD1. An excess of VLCFAs disrupts essential …

Mitochondrial dysfunction and oxidative damage cooperatively fuel axonal degeneration in X-linked adrenoleukodystrophy

S Fourcade, J López-Erauskin, M Ruiz, I Ferrer, A Pujol - Biochimie, 2014 - Elsevier
Abstract X-linked adrenoleukodystrophy (X-ALD) is the most frequent inherited monogenic
demyelinating disease (minimal incidence 1: 17,000). It is often lethal and currently lacks a …

[HTML][HTML] LDHB deficiency promotes mitochondrial dysfunction mediated oxidative stress and neurodegeneration in adult mouse brain

JS Park, K Saeed, MH Jo, MW Kim, HJ Lee, CB Park… - Antioxidants, 2022 - mdpi.com
Age-related decline in mitochondrial function and oxidative stress plays a critical role in
neurodegeneration. Lactate dehydrogenase-B (LDHB) is a glycolytic enzyme that catalyzes …

Progressive alterations in amino acid and lipid metabolism correlate with peripheral neuropathy in PolgD257A mice

EW Lim, MK Handzlik, E Trefts, JM Gengatharan… - Science …, 2021 - science.org
Mitochondria are central to metabolic homeostasis, and progressive mitochondrial defects
have diverse metabolic consequences that could drive distinct pathophysiological states …

[HTML][HTML] Inhibition of HIF-prolyl-4-hydroxylases prevents mitochondrial impairment and cell death in a model of neuronal oxytosis

S Neitemeier, AM Dolga, B Honrath… - Cell death & …, 2016 - nature.com
Mitochondrial impairment induced by oxidative stress is a main characteristic of intrinsic cell
death pathways in neurons underlying the pathology of neurodegenerative diseases …

Oxidative stress modulates mitochondrial failure and cyclophilin D function in X-linked adrenoleukodystrophy

J Lopez-Erauskin, J Galino, P Bianchi, S Fourcade… - Brain, 2012 - academic.oup.com
A common process associated with oxidative stress and severe mitochondrial impairment is
the opening of the mitochondrial permeability transition pore, as described in many …

The nuclear cofactor receptor interacting protein-140 (RIP140) regulates the expression of genes involved in Aβ generation

K Blondrath, JH Steel, L Katsouri, M Ries… - Neurobiology of …, 2016 - Elsevier
The receptor interacting protein-140 (RIP140) is a cofactor for several nuclear receptors and
has been involved in the regulation of metabolic and inflammatory genes. We hypothesize …