Beneficial Effects of the Direct AMP-Kinase Activator PXL770 in In Vitro and In Vivo Models of X-Linked Adrenoleukodystrophy

Efficacy and safety of PXL770, a direct AMP kinase activator, for the treatment of non-alcoholic fatty liver disease (STAMP-NAFLD): a randomised, double-blind, placebo-controlled, phase 2a study

(2021) Kenneth Cusi et al.   Lancet Gastroenterology & Hepatology

X‐linked adrenoleukodystrophy: Pathology, pathophysiology, diagnostic testing, newborn screening and therapies

(2020) Bela R. Turk et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Estimating the relative frequency of leukodystrophies and recommendations for carrier screening in the era of next‐generation sequencing

(2020) Johanna L. Schmidt et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Cellular and Molecular Mechanisms of Metformin Action

(2020) Traci E LaMoia et al.   ENDOCRINE REVIEWS

Defining diagnostic cutoffs in neurological patients for serum very long chain fatty acids (VLCFA) in genetically confirmed X-Adrenoleukodystrophy

(2020) Tim W. Rattay et al.   Scientific Reports

AMPK‑SIRT1 pathway dysfunction contributes to neuron apoptosis and cognitive impairment induced by sevoflurane

(2020) Liwei Liu et al.   Molecular Medicine Reports

AMP-activated protein kinase: the current landscape for drug development

(2019) Gregory R. Steinberg et al.   NATURE REVIEWS DRUG DISCOVERY

Metformin Improves Mitochondrial Respiratory Activity through Activation of AMPK

(2019) Yu Wang et al.   Cell Reports

A Thyroid Hormone–Based Strategy for Correcting the Biochemical Abnormality in X-Linked Adrenoleukodystrophy

(2017) Meredith D. Hartley et al.   ENDOCRINOLOGY

Inflammatory profile in X-linked adrenoleukodystrophy patients: Understanding disease progression

(2017) Desirèe Padilha Marchetti et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

AMPK: guardian of metabolism and mitochondrial homeostasis

(2017) Sébastien Herzig et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Heat shock protein expression in cerebral X-linked adrenoleukodystrophy reveals astrocyte stress prior to myelin loss

(2017) A. L. Görtz et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Metformin-induced mitochondrial function and ABCD2 up-regulation in X-linked adrenoleukodystrophy involves AMP-activated protein kinase

(2016) Jaspreet Singh et al.   JOURNAL OF NEUROCHEMISTRY

The ABCD's of 5′-adenosine monophosphate-activated protein kinase and adrenoleukodystrophy

(2016) Ian Weidling et al.   JOURNAL OF NEUROCHEMISTRY

Adrenoleukodystrophy – neuroendocrine pathogenesis and redefinition of natural history

(2016) Stephan Kemp et al.   Nature Reviews Endocrinology

Loss of AMP-Activated Protein Kinase Induces Mitochondrial Dysfunction and Proinflammatory Response in Unstimulated Abcd1-Knockout Mice Mixed Glial Cells

(2015) Jaspreet Singh et al.   MEDIATORS OF INFLAMMATION

Loss of AMP-activated protein kinase in X-linked adrenoleukodystrophy patient-derived fibroblasts and lymphocytes

(2014) Jaspreet Singh et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Mitochondrial dysfunction and oxidative damage cooperatively fuel axonal degeneration in X-linked adrenoleukodystrophy

(2013) Stéphane Fourcade et al.   BIOCHIMIE

Pathophysiology of X-linked adrenoleukodystrophy

(2013) J. Berger et al.   BIOCHIMIE

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

(2013) Laia Morató et al.   BRAIN

X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism is severely impaired in monocytes but not in lymphocytes

(2013) F. D. Weber et al.   HUMAN MOLECULAR GENETICS

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

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

Biguanides suppress hepatic glucagon signalling by decreasing production of cyclic AMP

(2013) Russell A. Miller et al.   NATURE

X-linked adrenoleukodystrophy: Clinical, metabolic, genetic and pathophysiological aspects

(2012) Stephan Kemp et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

X-linked adrenoleukodystrophy (X-ALD): clinical presentation and guidelines for diagnosis, follow-up and management

(2012) Marc Engelen et al.   Orphanet Journal of Rare Diseases

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

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

Pathomechanisms Underlying X-Adrenoleukodystrophy: A Three-Hit Hypothesis

(2010) Inderjit Singh et al.   BRAIN PATHOLOGY

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

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

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