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

Peroxisome biogenesis in mammalian cells: The impact of genes and environment

(2016) Rebecca L. Farr et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Hepatic dysfunction in peroxisomal disorders

(2016) Myriam Baes et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Peroxisomes in brain development and function

(2016) Johannes Berger et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Human disorders of peroxisome metabolism and biogenesis

(2016) Hans R. Waterham et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Peroxisome biogenesis disorders in the Zellweger spectrum: An overview of current diagnosis, clinical manifestations, and treatment guidelines

(2016) Nancy E. Braverman et al.   MOLECULAR GENETICS AND METABOLISM

Ursodeoxycholic Acid (UDCA) Exerts Anti-Atherogenic Effects by Inhibiting RAGE Signaling in Diabetic Atherosclerosis

(2016) Jihwa Chung et al.   PLoS One

The unfolded protein response in neurodegenerative diseases: a neuropathological perspective

(2015) Wiep Scheper et al.   ACTA NEUROPATHOLOGICA

Peroxisomal Disorders: A Review on Cerebellar Pathologies

(2015) Stephanie De Munter et al.   BRAIN PATHOLOGY

Activation of sirtuin 1 as therapy for the peroxisomal disease adrenoleukodystrophy

(2015) L Morató et al.   CELL DEATH AND DIFFERENTIATION

Lipid-dependent regulation of the unfolded protein response

(2015) Romain Volmer et al.   CURRENT OPINION IN CELL BIOLOGY

The TUDCA trial - innovative trial designs for amyotrophic lateral sclerosis drugs?

(2015) A. C. Ludolph   EUROPEAN JOURNAL OF NEUROLOGY

Tauroursodeoxycholic acid in the treatment of patients with amyotrophic lateral sclerosis

(2015) A. E. Elia et al.   EUROPEAN JOURNAL OF NEUROLOGY

Oxidative stress, mitochondrial and proteostasis malfunction in adrenoleukodystrophy: A paradigm for axonal degeneration

(2015) Stéphane Fourcade et al.   FREE RADICAL BIOLOGY AND MEDICINE

Abnormalities of motor function, transcription and cerebellar structure in mouse models ofTHAP1dystonia

(2015) Marta Ruiz et al.   HUMAN MOLECULAR GENETICS

Amyloid-β pathology is attenuated by tauroursodeoxycholic acid treatment in APP/PS1 mice after disease onset

(2015) Pedro A. Dionísio et al.   NEUROBIOLOGY OF AGING

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

(2014) Yong-Jie Zhang et al.   ACTA NEUROPATHOLOGICA

Autophagy induction halts axonal degeneration in a mouse model of X-adrenoleukodystrophy

(2014) Nathalie Launay et al.   ACTA NEUROPATHOLOGICA

Hif-2α Promotes Degradation of Mammalian Peroxisomes by Selective Autophagy

(2014) Katharina M. Walter et al.   Cell Metabolism

Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases

(2014) Claudio Hetz et al.   NATURE REVIEWS NEUROSCIENCE

Granulovacuolar degeneration and unfolded protein response in mouse models of tauopathy and Aβ amyloidosis

(2014) Christoph Köhler et al.   NEUROBIOLOGY OF DISEASE

Activation of the endoplasmic reticulum unfolded protein response by lipid disequilibrium without disturbed proteostasis in vivo

(2014) N. S. Hou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Peroxisome Formation and Maintenance Are Dependent on the Endoplasmic Reticulum

(2013) Henk F. Tabak et al.   Annual Review of Biochemistry

Recent advances in the application of metabolomics to Alzheimer's Disease

(2013) Eugenia Trushina et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Brain peroxisomes

(2013) D. Trompier et al.   BIOCHIMIE

Cholesterol biosynthesis and ER stress in peroxisome deficiency

(2013) Phyllis L. Faust et al.   BIOCHIMIE

Oxidative stress regulates the ubiquitin–proteasome system and immunoproteasome functioning in a mouse model of X-adrenoleukodystrophy

(2013) Nathalie Launay et al.   BRAIN

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

(2013) Laia Morató et al.   BRAIN

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

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

Glutathione imbalance in patients with X-linked adrenoleukodystrophy

(2013) Sara Petrillo et al.   MOLECULAR GENETICS AND METABOLISM

Plasma Ceramide and Glucosylceramide Metabolism Is Altered in Sporadic Parkinson's Disease and Associated with Cognitive Impairment: A Pilot Study

(2013) Michelle M. Mielke et al.   PLoS One

Membrane lipid saturation activates endoplasmic reticulum unfolded protein response transducers through their transmembrane domains

(2013) R. Volmer et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Peroxisome deficiency-induced ER stress and SREBP-2 pathway activation in the liver of newborn PEX2 knock-out mice

(2012) Werner J. Kovacs et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Mouse models for peroxisome biogenesis defects and β-oxidation enzyme deficiencies

(2012) Myriam Baes et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy

(2012) Rene L. Vidal et al.   HUMAN MOLECULAR GENETICS

Accumulation of Toxic  -Synuclein Oligomer within Endoplasmic Reticulum Occurs in  -Synucleinopathy In Vivo

(2012) E. Colla et al.   JOURNAL OF NEUROSCIENCE

Endoplasmic Reticulum Stress Is Important for the Manifestations of  -Synucleinopathy In Vivo

(2012) E. Colla et al.   JOURNAL OF NEUROSCIENCE

Saturated fatty acid induction of endoplasmic reticulum stress and apoptosis in human liver cells via the PERK/ATF4/CHOP signaling pathway

(2012) Jie Cao et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

TUDCA, a Bile Acid, Attenuates Amyloid Precursor Protein Processing and Amyloid-β Deposition in APP/PS1 Mice

(2012) Ana F. Nunes et al.   MOLECULAR NEUROBIOLOGY

Tauroursodeoxycholic Acid Prevents MPTP-Induced Dopaminergic Cell Death in a Mouse Model of Parkinson’s Disease

(2012) M. Castro-Caldas et al.   MOLECULAR NEUROBIOLOGY

Life-or-Death Decisions upon Axonal Damage

(2012) Francesco Roselli et al.   NEURON

Differential Effects of Unfolded Protein Response Pathways on Axon Injury-Induced Death of Retinal Ganglion Cells

(2012) Yang Hu et al.   NEURON

Progressive Endoplasmic Reticulum Stress Contributes to Hepatocarcinogenesis in Fatty Acyl-CoA Oxidase 1–Deficient Mice

(2011) Jiansheng Huang et al.   AMERICAN JOURNAL OF PATHOLOGY

Antioxidants halt axonal degeneration in a mouse model of X-adrenoleukodystrophy

(2011) Jone López-Erauskin 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

Mitochondrial Dysfunction and Oxidative and Endoplasmic Reticulum Stress in Argyrophilic Grain Disease

(2011) Ekaterina V. Ilieva et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

The eIF2 kinase PERK and the integrated stress response facilitate activation of ATF6 during endoplasmic reticulum stress

(2011) Brian F. Teske et al.   MOLECULAR BIOLOGY OF THE CELL

Aberrant lipid metabolism disrupts calcium homeostasis causing liver endoplasmic reticulum stress in obesity

(2011) Suneng Fu et al.   NATURE

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Identification of microRNAs during rat liver regeneration after partial hepatectomy and modulation by ursodeoxycholic acid

(2010) Rui E. Castro et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Pathomechanisms Underlying X-Adrenoleukodystrophy: A Three-Hit Hypothesis

(2010) Inderjit Singh et al.   BRAIN PATHOLOGY

General Aspects and Neuropathology of X-Linked Adrenoleukodystrophy

(2010) Isidro Ferrer et al.   BRAIN PATHOLOGY

Current and Future Pharmacological Treatment Strategies in X-Linked Adrenoleukodystrophy

(2010) Johannes Berger et al.   BRAIN PATHOLOGY

Safety, Tolerability, and Cerebrospinal Fluid Penetration of Ursodeoxycholic Acid in Patients With Amyotrophic Lateral Sclerosis

(2010) Gareth J. Parry et al.   CLINICAL NEUROPHARMACOLOGY

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

(2010) Rob Ofman et al.   EMBO Molecular Medicine

Depletion of oxidative and endoplasmic reticulum stress regulators in Pick disease

(2010) Ekaterina V. Ilieva et al.   FREE RADICAL BIOLOGY AND MEDICINE

Valproic acid induces antioxidant effects in X-linked adrenoleukodystrophy

(2010) Stéphane Fourcade et al.   HUMAN MOLECULAR GENETICS

Regulation of basal cellular physiology by the homeostatic unfolded protein response

(2010) D. Thomas Rutkowski et al.   JOURNAL OF CELL BIOLOGY

Endoplasmic Reticulum Stress in Motor Neurons of the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis

(2010) Shoichi Sasaki   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

The Unfolded Protein Response Is Activated in Pretangle Neurons in Alzheimer's Disease Hippocampus

(2009) Jeroen J.M. Hoozemans et al.   AMERICAN JOURNAL OF PATHOLOGY

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

Endoplasmic Reticulum Stress Plays a Central Role in Development of Leptin Resistance

(2009) Lale Ozcan et al.   Cell Metabolism

Cerebrospinal Fluid Steroidomics: Are Bioactive Bile Acids Present in Brain?

(2009) Michael Ogundare et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Bile acids: the role of peroxisomes

(2009) Sacha Ferdinandusse et al.   JOURNAL OF LIPID RESEARCH

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

(2009) N. Cartier et al.   SCIENCE

A key role for the peroxisomal ABCD2 transporter in fatty acid homeostasis

(2008) Stéphane Fourcade et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

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

Peroxisome Deficiency Causes a Complex Phenotype because of Hepatic SREBP/Insig Dysregulation Associated with Endoplasmic Reticulum Stress

(2008) Werner J. Kovacs et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Endoplasmic Reticulum Quality Control and the Unfolded Protein Response: Insights from Plants

(2008) Alessandro Vitale et al.   TRAFFIC

Activation and Dysregulation of the Unfolded Protein Response in Nonalcoholic Fatty Liver Disease

(2007) Puneet Puri et al.   GASTROENTEROLOGY