Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model
Aims Mitochondrial dysfunction and inflammation are at the core of axonal degeneration in
several multifactorial neurodegenerative diseases, including multiple sclerosis, Alzheimer's …
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 …
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 …
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 …
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
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 …
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
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 …
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 …
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 …
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 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
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 …
has been involved in the regulation of metabolic and inflammatory genes. We hypothesize …