ABCD1 deletion-induced mitochondrial dysfunction is corrected by SAHA: implication for adrenoleukodystrophy

Metabolism of Very Long-Chain Fatty Acids: Genes and Pathophysiology

(2014) Takayuki Sassa et al.   Biomolecules & Therapeutics

Caffeic acid phenethyl ester induces adrenoleukodystrophy (Abcd2) gene in human X-ALD fibroblasts and inhibits the proinflammatory response in Abcd1/2 silenced mouse primary astrocytes

(2013) Jaspreet Singh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

LXR antagonists induce ABCD2 expression

(2013) Catherine Gondcaille et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy

(2013) Laia Morató et al.   BRAIN

Mitochondrial Diseases of the Brain

(2013) Rajnish K. Chaturvedi et al.   FREE RADICAL BIOLOGY AND MEDICINE

Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy

(2013) J. López-Erauskin et al.   HUMAN MOLECULAR GENETICS

Histone Deacetylase Inhibitor Upregulates Peroxisomal Fatty Acid Oxidation and Inhibits Apoptotic Cell Death in Abcd1-Deficient Glial Cells

(2013) Jaspreet Singh et al.   PLoS One

Intrinsic acyl-CoA thioesterase activity of a peroxisomal ATP binding cassette transporter is required for transport and metabolism of fatty acids

(2013) Carine De Marcos Lousa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Oxidative stress modulates mitochondrial failure and cyclophilin D function in X-linked adrenoleukodystrophy

(2012) Jone López-Erauskin et al.   BRAIN

OXPHOS mutations and neurodegeneration

(2012) Werner J H Koopman et al.   EMBO JOURNAL

Assessment of drug-induced mitochondrial dysfunction via altered cellular respiration and acidification measured in a 96-well platform

(2012) Sashi Nadanaciva et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Peroxisomal Fatty Acid Uptake Mechanism inSaccharomyces cerevisiae

(2012) Carlo W. T. van Roermund et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Evidence of oxidative stress in very long chain fatty acid – Treated oligodendrocytes and potentialization of ROS production using RNA interference-directed knockdown of ABCD1 and ACOX1 peroxisomal proteins

(2012) M. Baarine et al.   NEUROSCIENCE

Induction of Mitochondrial Changes Associated with Oxidative Stress on Very Long Chain Fatty Acids (C22:0, C24:0, or C26:0)-Treated Human Neuronal Cells (SK-NB-E)

(2012) Amira Zarrouk et al.   Oxidative Medicine and Cellular Longevity

Induced pluripotent stem cell models from X-linked adrenoleukodystrophy patients

(2011) Jiho Jang et al.   ANNALS OF NEUROLOGY

Oxidative Damage Compromises Energy Metabolism in the Axonal Degeneration Mouse Model of X-Adrenoleukodystrophy

(2011) Jorge Galino et al.   ANTIOXIDANTS & REDOX SIGNALING

Bioenergetic defect associated with mKATP channel opening in a mouse model carrying a mitofusin 2 mutation

(2011) Virginie Guillet et al.   FASEB JOURNAL

Functional genomic analysis unravels a metabolic-inflammatory interplay in adrenoleukodystrophy

(2011) Agatha Schlüter et al.   HUMAN MOLECULAR GENETICS

HDAC inhibitor SAHA normalizes the levels of VLCFAs in human skin fibroblasts from X-ALD patients and downregulates the expression of proinflammatory cytokines in Abcd1/2-silenced mouse astrocytes

(2011) Jaspreet Singh et al.   JOURNAL OF LIPID RESEARCH

Incidence of Abcd1 level on the induction of cell death and organelle dysfunctions triggered by very long chain fatty acids and TNF-α on oligodendrocytes and astrocytes

(2011) Mauhamad Baarine et al.   NEUROTOXICOLOGY

Evidence of Oxidative Stress in Alzheimer's Disease Brain and Antioxidant Therapy

(2010) Domenico Praticò   Annals of the New York Academy of Sciences

Evidence of Oxidant Damage in Huntington's Disease: Translational Strategies Using Antioxidants

(2010) Edward C. Stack et al.   Annals of the New York Academy of Sciences

Oxidative Stress in Parkinson's Disease

(2010) Chun Zhou et al.   Annals of the New York Academy of Sciences

Pathomechanisms Underlying X-Adrenoleukodystrophy: A Three-Hit Hypothesis

(2010) Inderjit Singh et al.   BRAIN PATHOLOGY

Accumulation and defective β-oxidation of very long chain fatty acids in Zellweger's syndrome, adrenoleukodystrophy and Refsum's disease variants

(2010) A. Poulos et al.   CLINICAL GENETICS

PEX13 deficiency in mouse brain as a model of Zellweger syndrome: abnormal cerebellum formation, reactive gliosis and oxidative stress

(2010) C. C. Muller et al.   Disease Models & Mechanisms

The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy

(2010) Rob Ofman et al.   EMBO Molecular Medicine

Valproic acid induces antioxidant effects in X-linked adrenoleukodystrophy

(2010) Stéphane Fourcade et al.   HUMAN MOLECULAR GENETICS

Very long-chain fatty acid accumulation causes lipotoxic response via 5-lipoxygenase in cerebral adrenoleukodystrophy

(2010) Mushfiquddin Khan et al.   JOURNAL OF LIPID RESEARCH

ELOVL1 production of C24 acyl-CoAs is linked to C24 sphingolipid synthesis

(2010) Y. Ohno et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Insulin-like growth factor-1 and neurotrophin-3 gene therapy prevents motor decline in an X-linked adrenoleukodystrophy mouse model

(2009) Roberto Mastroeni et al.   ANNALS OF NEUROLOGY

Protein Targets of Oxidative Damage in Human Neurodegenerative Diseases with Abnormal Protein Aggregates

(2009) Anna Martínez et al.   BRAIN PATHOLOGY

Inhibition of Histone Deacetylases: A Pharmacological Approach to the Treatment of Non-Cancer Disorders

(2009) Norbert Wiech et al.   CURRENT TOPICS IN MEDICINAL CHEMISTRY

Oxidative Imbalance in Nonstimulated X-Adrenoleukodystrophy-Derived Lymphoblasts

(2009) Takuhiro Uto et al.   DEVELOPMENTAL NEUROSCIENCE

Peroxisomal Dysfunction in Inflammatory Childhood White Matter Disorders: An Unexpected Contributor to Neuropathology

(2009) Inderjit Singh et al.   JOURNAL OF CHILD NEUROLOGY

Peroxisomal and mitochondrial status of two murine oligodendrocytic cell lines (158N, 158JP): potential models for the study of peroxisomal disorders associated with dysmyelination processes

(2009) Mauhamad Baarine et al.   JOURNAL OF NEUROCHEMISTRY

Adenine nucleotide translocase is involved in a mitochondrial coupling defect in MFN2-related Charcot–Marie–Tooth type 2A disease

(2009) Virginie Guillet et al.   NEUROGENETICS

Hematopoietic Stem Cell Gene Therapy with a Lentiviral Vector in X-Linked Adrenoleukodystrophy

(2009) N. Cartier et al.   SCIENCE

Is microglial apoptosis an early pathogenic change in cerebral X-linked adrenoleukodystrophy?

(2008) Florian S. Eichler et al.   ANNALS OF NEUROLOGY

Fatty liver and insulin resistance in obese Zucker rats: No role for mitochondrial dysfunction

(2008) Mélissa Flamment et al.   BIOCHIMIE

The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl-CoA esters

(2008) Carlo W. T. van Roermund et al.   FASEB JOURNAL

Toxic effects of X-linked adrenoleukodystrophy-associated, very long chain fatty acids on glial cells and neurons from rat hippocampus in culture

(2008) S. Hein et al.   HUMAN MOLECULAR GENETICS

Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy

(2008) S. Fourcade et al.   HUMAN MOLECULAR GENETICS

Silencing of Abcd1 and Abcd2 genes sensitizes astrocytes for inflammation: implication for X-adrenoleukodystrophy

(2008) Jaspreet Singh et al.   JOURNAL OF LIPID RESEARCH

Lipopolysaccharide-induced peroxisomal dysfunction exacerbates cerebral white matter injury: Attenuation by N-acetyl cysteine

(2007) Manjeet K. Paintlia et al.   EXPERIMENTAL NEUROLOGY