Axonal degeneration during aging and its functional role in neurodegenerative disorders

N Salvadores, M Sanhueza, P Manque… - Frontiers in …, 2017 - frontiersin.org
Aging constitutes the main risk factor for the development of neurodegenerative diseases.
This represents a major health issue worldwide that is only expected to escalate due to the …

How the brain fights fatty acids' toxicity

P Schönfeld, G Reiser - Neurochemistry International, 2021 - Elsevier
Neurons spurn hydrogen-rich fatty acids for energizing oxidative ATP synthesis, contrary to
other cells. This feature has been mainly attributed to a lower yield of ATP per reduced …

Peroxisomal ABC transporters: an update

A Tawbeh, C Gondcaille, D Trompier… - International Journal of …, 2021 - mdpi.com
ATP-binding cassette (ABC) transporters constitute one of the largest superfamilies of
conserved proteins from bacteria to mammals. In humans, three members of this family are …

Nuclear factor erythroid-2-related factor 2 signaling in the neuropathophysiology of inherited metabolic disorders

B Seminotti, M Grings, P Tucci, G Leipnitz… - Frontiers in Cellular …, 2021 - frontiersin.org
Inherited metabolic disorders (IMDs) are rare genetic conditions that affect multiple organs,
predominantly the central nervous system. Since treatment for a large number of IMDs is …

FDA-approved kinase inhibitors in preclinical and clinical trials for neurological disorders

A Lui, J Vanleuven, D Perekopskiy, D Liu, D Xu… - Pharmaceuticals, 2022 - mdpi.com
Cancers and neurological disorders are two major types of diseases. We previously
developed a new concept termed “Aberrant Cell Cycle Diseases”(ACCD), revealing that …

[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 …

Biomarker identification, safety, and efficacy of high-dose antioxidants for adrenomyeloneuropathy: a phase II pilot study

C Casasnovas, M Ruiz, A Schlüter, A Naudí… - …, 2019 - Springer
Abstract X-Adrenoleukodystrophy (X-ALD) and its adult-onset, most prevalent variant
adrenomyeloneuropathy (AMN) are caused by mutations in the peroxisomal transporter of …

CRISPR/Cas9-mediated knockout of Abcd1 and Abcd2 genes in BV-2 cells: novel microglial models for X-linked Adrenoleukodystrophy

Q Raas, C Gondcaille, Y Hamon, V Leoni… - … et Biophysica Acta (BBA …, 2019 - Elsevier
Abstract X-linked adrenoleukodystrophy (X-ALD), the most frequent peroxisomal disorder, is
associated with mutation in the ABCD1 gene which encodes a peroxisomal ATP-binding …

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 …

Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy

N Launay, M Ruiz, L Grau, FJ Ortega, EV Ilieva… - Acta …, 2017 - Springer
The activation of the highly conserved unfolded protein response (UPR) is prominent in the
pathogenesis of the most prevalent neurodegenerative disorders, such as Alzheimer's …