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] Targeting demyelination and virtual hypoxia with high-dose biotin as a treatment for progressive multiple sclerosis

F Sedel, D Bernard, DM Mock, A Tourbah - Neuropharmacology, 2016 - Elsevier
Progressive multiple sclerosis (MS) is a severely disabling neurological condition, and an
effective treatment is urgently needed. Recently, high-dose biotin has emerged as a …

Biotin rescues mitochondrial dysfunction and neurotoxicity in a tauopathy model

KM Lohr, B Frost, C Scherzer… - Proceedings of the …, 2020 - National Acad Sciences
Mitochondrial and metabolic dysfunction are often implicated in neurological disease, but
effective mechanism-based therapies remain elusive. We performed a genome-scale …

[HTML][HTML] Effects of biotin on survival, ensheathment, and ATP production by oligodendrocyte lineage cells in vitro

QL Cui, YH Lin, YKT Xu, MGF Fernandes, VTS Rao… - PLoS …, 2020 - journals.plos.org
Mechanisms implicated in disease progression in multiple sclerosis include continued
oligodendrocyte (OL)/myelin injury and failure of myelin repair. Underlying causes include …

[HTML][HTML] High dose pharmaceutical grade biotin (MD1003) accelerates differentiation of murine and grafted human oligodendrocyte progenitor cells in vivo

MJF Levy, B Garcia-Diaz, F Sedel… - International Journal of …, 2022 - mdpi.com
Accumulating evidences suggest a strong correlation between metabolic changes and
neurodegeneration in CNS demyelinating diseases such as multiple sclerosis (MS). Biotin …

Biotin attenuation of oxidative stress, mitochondrial dysfunction, lipid metabolism alteration and 7β-hydroxycholesterol-induced cell death in 158N murine …

R Sghaier, A Zarrouk, T Nury, I Badreddine… - Free radical …, 2019 - Taylor & Francis
Mitochondrial dysfunction and oxidative stress are involved in neurodegenerative diseases
associated with an enhancement of lipid peroxidation products such as 7β …

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 …

High-dose biotin for multiple sclerosis: A systematic review and meta-analyses of randomized controlled trials

AI Espiritu, PPM Remalante-Rayco - Multiple Sclerosis and Related …, 2021 - Elsevier
Background Biotin may activate the acetyl-CoA-, 3-methylcrotonyl-CoA-, propionyl-CoA-,
and pyruvate carboxylases to increase myelin repair and/or synthesis, and may enhance the …

Biotinidase knockout mice show cellular energy deficit and altered carbon metabolism gene expression similar to that of nutritional biotin deprivation: clues for the …

A Hernandez-Vazquez, B Wolf, K Pindolia… - Molecular Genetics and …, 2013 - Elsevier
Biotin is the prosthetic group of carboxylases that have important roles in the metabolism of
glucose, fatty acids and amino acids. Biotinidase has a key role in the reutilization of the …

Astroglial biotin deprivation under endoplasmic reticulum stress uncouples BCAA‐mTORC1 role in lipid synthesis to prolong autophagy inhibition in the aging brain

D Ganesan, A Ramaian Santhaseela… - Journal of …, 2020 - Wiley Online Library
Autophagy delays the onset of endoplasmic reticulum (ER) stress by recycling cellular
debris. However, the cues that elicit autophagy under the emergence of ER stress and their …