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 …
Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy
S Fourcade, J Lopez-Erauskin, J Galino… - Human molecular …, 2008 - academic.oup.com
Abstract X-linked adrenoleukodystrophy (X-ALD) is a fatal neurodegenerative disorder,
characterized by progressive cerebral demyelination cerebral childhood …
characterized by progressive cerebral demyelination cerebral childhood …
Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy
Abstract X-linked adrenoleukodystrophy (X-ALD) is an inherited metabolic disorder of the
nervous system characterized by axonopathy in spinal cords and/or cerebral demyelination …
nervous system characterized by axonopathy in spinal cords and/or cerebral demyelination …
[HTML][HTML] Oxidative stress underlying axonal degeneration in adrenoleukodystrophy: a paradigm for multifactorial neurodegenerative diseases?
X-linked adrenoleukodystrophy (X-ALD) is an inherited neurodegenerative disorder
expressed as four disease variants characterized by adrenal insufficiency and graded …
expressed as four disease variants characterized by adrenal insufficiency and graded …
[HTML][HTML] Pathophysiology of X-linked adrenoleukodystrophy
J Berger, S Forss-Petter, FS Eichler - Biochimie, 2014 - Elsevier
Currently the molecular basis for the clinical heterogeneity of X-linked
adrenoleukodystrophy (X-ALD) is poorly understood. The genetic bases for all different …
adrenoleukodystrophy (X-ALD) is poorly understood. The genetic bases for all different …
Adreno-leukodystrophy: oxidative stress of mice and men
JM Powers, Z Pei, AK Heinzer, R Deering… - … of Neuropathology & …, 2005 - academic.oup.com
X-linked adreno-leukodystrophy is a progressive, systemic peroxisomal disorder that affects
primarily nervous system myelin and axons as well as the adrenal cortex. Several divergent …
primarily nervous system myelin and axons as well as the adrenal cortex. Several divergent …
Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy
X-linked adrenoleukodystrophy is a neurometabolic disorder caused by inactivation of the
peroxisomal ABCD1 transporter of very long-chain fatty acids. In mice, ABCD1 loss causes …
peroxisomal ABCD1 transporter of very long-chain fatty acids. In mice, ABCD1 loss causes …
[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 …
Novel therapeutic targets and drug candidates for modifying disease progression in adrenoleukodystrophy
A Pujol - Advanced Therapies in Pediatric Endocrinology and …, 2016 - karger.com
X-linked adrenoleukodystrophy (X-ALD) is the most frequent inherited monogenic
demyelinating disease. It is often lethal and currently lacks a satisfactory therapy. The …
demyelinating disease. It is often lethal and currently lacks a satisfactory therapy. The …
ABCD1 and X‐linked adrenoleukodystrophy: A disease with a markedly variable phenotype showing conserved neurobiology in animal models
J Manor, H Chung, PK Bhagwat… - Journal of …, 2021 - Wiley Online Library
Abstract X‐linked adrenoleukodystrophy (X‐ALD) is a phenotypically heterogeneous
disorder involving defective peroxisomal β‐oxidation of very long‐chain fatty acids …
disorder involving defective peroxisomal β‐oxidation of very long‐chain fatty acids …