Selenoprotein V protects against endoplasmic reticulum stress and oxidative injury induced by pro-oxidants

X Zhang, W Xiong, LL Chen, JQ Huang… - Free Radical Biology and …, 2020 - Elsevier
Selenoprotein V (SELENOV) contains a thioredoxin-like fold and a conserved CxxU motif
with a potential redox function. This study was to assess its in vivo and in vitro roles and …

The peroxisomal fatty acid transporter ABCD1/PMP-4 is required in the C. elegans hypodermis for axonal maintenance: A worm model for adrenoleukodystrophy

A Coppa, S Guha, S Fourcade, J Parameswaran… - Free Radical Biology …, 2020 - Elsevier
Adrenoleukodystrophy is a neurometabolic disorder caused by a defective peroxisomal
ABCD1 transporter of very long-chain fatty acids (VLCFAs). Its pathogenesis is incompletely …

High‐dose biotin restores redox balance, energy and lipid homeostasis, and axonal health in a model of adrenoleukodystrophy

S Fourcade, L Goicoechea, J Parameswaran… - Brain …, 2020 - Wiley Online Library
Biotin is an essential cofactor for carboxylases that regulates the energy metabolism.
Recently, high‐dose pharmaceutical‐grade biotin (MD1003) was shown to improve clinical …

[HTML][HTML] Docosahexaenoic acid attenuates mitochondrial alterations and oxidative stress leading to cell death induced by very long-chain fatty acids in a mouse …

T Nury, M Doria, G Lizard, A Vejux - International Journal of Molecular …, 2020 - mdpi.com
In the case of neurodegenerative pathologies, the therapeutic arsenal available is often
directed towards the consequences of the disease. The purpose of this study is, therefore, to …

[HTML][HTML] Chronic Progressive Spastic Paraparesis: Think of Peroxisomal Disorders-A Case Report of X-Linked Adult Onset Adrenoleukodystrophy With an Update on …

H Kesserwani - Cureus, 2020 - ncbi.nlm.nih.gov
The etiology of chronic progressive myelopathy can be a challenging diagnostic dilemma,
especially in the absence of a structural lesion of the neural axis or a conspicuous …