Published in Acta Neuropathol on December 31, 2014
Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy. Acta Neuropathol (2016) 0.75
Axonal Degeneration during Aging and Its Functional Role in Neurodegenerative Disorders. Front Neurosci (2017) 0.75
Development by self-digestion: molecular mechanisms and biological functions of autophagy. Dev Cell (2004) 21.68
Loss of autophagy in the central nervous system causes neurodegeneration in mice. Nature (2006) 21.40
Methods in mammalian autophagy research. Cell (2010) 20.08
p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death. J Cell Biol (2005) 18.23
Promotion of tumorigenesis by heterozygous disruption of the beclin 1 autophagy gene. J Clin Invest (2003) 16.67
Beclin 1, an autophagy gene essential for early embryonic development, is a haploinsufficient tumor suppressor. Proc Natl Acad Sci U S A (2003) 15.59
Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat Genet (2004) 14.31
Autophagy regulates lipid metabolism. Nature (2009) 13.33
Dissection of the autophagosome maturation process by a novel reporter protein, tandem fluorescent-tagged LC3. Autophagy (2007) 11.74
Autophagy and the integrated stress response. Mol Cell (2010) 11.21
Protein degradation and protection against misfolded or damaged proteins. Nature (2003) 10.19
Reactive oxygen species are essential for autophagy and specifically regulate the activity of Atg4. EMBO J (2007) 9.65
Hematopoietic stem cell gene therapy with a lentiviral vector in X-linked adrenoleukodystrophy. Science (2009) 8.80
The role of Atg proteins in autophagosome formation. Annu Rev Cell Dev Biol (2011) 8.18
Protein turnover via autophagy: implications for metabolism. Annu Rev Nutr (2007) 5.75
Autophagy gone awry in neurodegenerative diseases. Nat Neurosci (2010) 4.72
Autophagy induction and autophagosome clearance in neurons: relationship to autophagic pathology in Alzheimer's disease. J Neurosci (2008) 4.63
Reduced TOR signaling extends chronological life span via increased respiration and upregulation of mitochondrial gene expression. Cell Metab (2007) 4.59
The role of autophagy in neurodegenerative disease. Nat Med (2013) 4.46
In search of an "autophagomometer". Autophagy (2009) 4.31
Does bafilomycin A1 block the fusion of autophagosomes with lysosomes? Autophagy (2008) 3.47
Autophagy, mitochondria and oxidative stress: cross-talk and redox signalling. Biochem J (2012) 3.37
Induction of autophagy in axonal dystrophy and degeneration. J Neurosci (2006) 3.28
Autophagy and neurodegeneration: when the cleaning crew goes on strike. Lancet Neurol (2007) 3.18
Regulation of macroautophagy by mTOR and Beclin 1 complexes. Biochimie (2007) 2.95
Pathogenic lysosomal depletion in Parkinson's disease. J Neurosci (2010) 2.73
Rapamycin treatment augments motor neuron degeneration in SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Autophagy (2011) 2.47
Mitochondrial dysfunction and oxidative stress mediate the physiological impairment induced by the disruption of autophagy. Aging (Albany NY) (2009) 2.45
Altered lipid content inhibits autophagic vesicular fusion. FASEB J (2010) 2.35
Methamphetamine-induced degeneration of dopaminergic neurons involves autophagy and upregulation of dopamine synthesis. J Neurosci (2002) 2.31
X-linked adrenoleukodystrophy (X-ALD): clinical presentation and guidelines for diagnosis, follow-up and management. Orphanet J Rare Dis (2012) 2.28
Laforin, the most common protein mutated in Lafora disease, regulates autophagy. Hum Mol Genet (2010) 2.22
Changes in the proteolytic activities of proteasomes and lysosomes in human fibroblasts produced by serum withdrawal, amino-acid deprivation and confluent conditions. Biochem J (2003) 1.89
Mechanisms of acute axonal degeneration in the optic nerve in vivo. Proc Natl Acad Sci U S A (2010) 1.70
Lipophagy: connecting autophagy and lipid metabolism. Int J Cell Biol (2012) 1.69
Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3. Brain (2009) 1.66
Inducing autophagy by rapamycin before, but not after, the formation of plaques and tangles ameliorates cognitive deficits. PLoS One (2011) 1.62
Autophagy signaling through reactive oxygen species. Antioxid Redox Signal (2011) 1.52
The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl-CoA esters. FASEB J (2008) 1.51
Differential roles of unsaturated and saturated fatty acids on autophagy and apoptosis in hepatocytes. J Pharmacol Exp Ther (2011) 1.49
Oxidative stress and autophagy in cardiac disease, neurological disorders, aging and cancer. Oxid Med Cell Longev (2010) 1.48
The late stages of autophagy: how does the end begin? Cell Death Differ (2009) 1.47
General aspects and neuropathology of X-linked adrenoleukodystrophy. Brain Pathol (2010) 1.41
Reversal of early neurologic and neuroradiologic manifestations of X-linked adrenoleukodystrophy by bone marrow transplantation. N Engl J Med (1990) 1.41
Regulation of autophagy by mTOR-dependent and mTOR-independent pathways: autophagy dysfunction in neurodegenerative diseases and therapeutic application of autophagy enhancers. Biochem Soc Trans (2013) 1.37
Methodological considerations for assessing autophagy modulators: a study with calcium phosphate precipitates. Autophagy (2009) 1.36
Adreno-leukodystrophy: oxidative stress of mice and men. J Neuropathol Exp Neurol (2005) 1.34
Protein targets of oxidative damage in human neurodegenerative diseases with abnormal protein aggregates. Brain Pathol (2009) 1.33
Determination of protein carbonyl groups by immunoblotting. Anal Biochem (1999) 1.32
Antioxidants halt axonal degeneration in a mouse model of X-adrenoleukodystrophy. Ann Neurol (2011) 1.31
Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy. Hum Mol Genet (2008) 1.27
Current and future pharmacological treatment strategies in X-linked adrenoleukodystrophy. Brain Pathol (2010) 1.27
Autophagy activation and enhanced mitophagy characterize the Purkinje cells of pcd mice prior to neuronal death. Mol Brain (2009) 1.26
Autophagy as an essential cellular antioxidant pathway in neurodegenerative disease. Redox Biol (2013) 1.24
Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy. Hum Mol Genet (2004) 1.23
The mechanism and physiological function of macroautophagy. J Innate Immun (2013) 1.22
Free fatty acids stimulate autophagy in pancreatic β-cells via JNK pathway. Biochem Biophys Res Commun (2010) 1.21
Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy. Hum Mol Genet (2002) 1.21
Src-dependent impairment of autophagy by oxidative stress in a mouse model of Duchenne muscular dystrophy. Nat Commun (2014) 1.21
Pathomechanisms underlying X-adrenoleukodystrophy: a three-hit hypothesis. Brain Pathol (2010) 1.20
A mouse model for X-linked adrenoleukodystrophy. Proc Natl Acad Sci U S A (1997) 1.20
Induction of autophagy by palmitic acid via protein kinase C-mediated signaling pathway independent of mTOR (mammalian target of rapamycin). J Biol Chem (2012) 1.19
Interactions of a very long chain fatty acid with model membranes and serum albumin. Implications for the pathogenesis of adrenoleukodystrophy. J Clin Invest (1995) 1.18
Antioxidants can inhibit basal autophagy and enhance neurodegeneration in models of polyglutamine disease. Hum Mol Genet (2010) 1.15
The ubiquitin proteasome system in neurodegenerative diseases: culprit, accomplice or victim? Prog Neurobiol (2012) 1.13
Regulation of lysosomal autophagy in transformed and non-transformed mouse fibroblasts under several growth conditions. Exp Cell Res (1984) 1.11
A key role for the peroxisomal ABCD2 transporter in fatty acid homeostasis. Am J Physiol Endocrinol Metab (2008) 1.07
Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage. Hum Mol Genet (2005) 1.06
Valproic acid induces antioxidant effects in X-linked adrenoleukodystrophy. Hum Mol Genet (2010) 1.03
Genetic reduction of mammalian target of rapamycin ameliorates Alzheimer's disease-like cognitive and pathological deficits by restoring hippocampal gene expression signature. J Neurosci (2014) 1.00
Phase II study of temsirolimus (CCI-779) in women with recurrent, unresectable, locally advanced or metastatic carcinoma of the cervix. A trial of the NCIC Clinical Trials Group (NCIC CTG IND 199). Gynecol Oncol (2013) 0.99
Oxidative stress underlying axonal degeneration in adrenoleukodystrophy: a paradigm for multifactorial neurodegenerative diseases? Biochim Biophys Acta (2012) 0.99
Reduced mammalian target of rapamycin activity facilitates mitochondrial retrograde signaling and increases life span in normal human fibroblasts. Aging Cell (2013) 0.99
Autophagy, and BiP level decrease are early key events in retrograde degeneration of motoneurons. Cell Death Differ (2011) 0.99
Stress-induced alterations in autophagic pathway: relationship to ubiquitin system. Am J Physiol (1992) 0.97
ABCD2 is abundant in adipose tissue and opposes the accumulation of dietary erucic acid (C22:1) in fat. J Lipid Res (2010) 0.96
Phase II trial of temsirolimus alone and in combination with irinotecan for KRAS mutant metastatic colorectal cancer: outcome and results of KRAS mutational analysis in plasma. Acta Oncol (2013) 0.96
Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy. Hum Mol Genet (2013) 0.95
OPA1 deficiency associated with increased autophagy in retinal ganglion cells in a murine model of dominant optic atrophy. Invest Ophthalmol Vis Sci (2009) 0.93
Functional genomic analysis unravels a metabolic-inflammatory interplay in adrenoleukodystrophy. Hum Mol Genet (2011) 0.91
Impaired very long-chain acyl-CoA β-oxidation in human X-linked adrenoleukodystrophy fibroblasts is a direct consequence of ABCD1 transporter dysfunction. J Biol Chem (2013) 0.90
Annexin A5 stimulates autophagy and inhibits endocytosis. J Cell Sci (2012) 0.90
Oxidative damage compromises energy metabolism in the axonal degeneration mouse model of X-adrenoleukodystrophy. Antioxid Redox Signal (2011) 0.90
Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy. Brain (2013) 0.89
Acute NMDA toxicity in cultured rat cerebellar granule neurons is accompanied by autophagy induction and late onset autophagic cell death phenotype. BMC Neurosci (2010) 0.88
Prevention of neointimal proliferation by immunosuppression in synthetic vascular grafts. Eur J Cardiothorac Surg (2001) 0.88
X-linked adrenoleukodystrophy: defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters. Clin Chim Acta (1987) 0.84
Insulin-like growth factor-1 and neurotrophin-3 gene therapy prevents motor decline in an X-linked adrenoleukodystrophy mouse model. Ann Neurol (2009) 0.83
Mitochondrial dysfunction and oxidative damage cooperatively fuel axonal degeneration in X-linked adrenoleukodystrophy. Biochimie (2013) 0.81
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. Neurotoxicology (2011) 0.81
Oxidative stress regulates the ubiquitin-proteasome system and immunoproteasome functioning in a mouse model of X-adrenoleukodystrophy. Brain (2013) 0.80
Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes. Autophagy (2007) 20.92
Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy (2012) 20.08
Tissue-specific autophagy alterations and increased tumorigenesis in mice deficient in Atg4C/autophagin-3. J Biol Chem (2007) 4.22
Activation of chaperone-mediated autophagy during oxidative stress. Mol Biol Cell (2004) 3.26
Long non-coding antisense RNA controls Uchl1 translation through an embedded SINEB2 repeat. Nature (2012) 3.08
Antibiotic-induced SOS response promotes horizontal dissemination of pathogenicity island-encoded virulence factors in staphylococci. Mol Microbiol (2005) 2.42
High-flow oxygen therapy in acute respiratory failure. Respir Care (2010) 2.40
Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts. Mol Biol Cell (2004) 2.29
Laforin, the most common protein mutated in Lafora disease, regulates autophagy. Hum Mol Genet (2010) 2.22
A DNA methylation fingerprint of 1628 human samples. Genome Res (2011) 2.16
A myriad of miRNA variants in control and Huntington's disease brain regions detected by massively parallel sequencing. Nucleic Acids Res (2010) 1.99
MicroRNA profiling of Parkinson's disease brains identifies early downregulation of miR-34b/c which modulate mitochondrial function. Hum Mol Genet (2011) 1.92
Changes in the proteolytic activities of proteasomes and lysosomes in human fibroblasts produced by serum withdrawal, amino-acid deprivation and confluent conditions. Biochem J (2003) 1.89
Ki-67 proliferative index predicts clinical outcome in patients with atypical or anaplastic meningioma. Neuropathology (2007) 1.76
Bap-dependent biofilm formation by pathogenic species of Staphylococcus: evidence of horizontal gene transfer? Microbiology (2005) 1.68
Oxidative and endoplasmic reticulum stress interplay in sporadic amyotrophic lateral sclerosis. Brain (2007) 1.52
The phosphatase activity of laforin is dispensable to rescue Epm2a-/- mice from Lafora disease. Brain (2014) 1.43
Regulation of glycogen synthesis by the laforin-malin complex is modulated by the AMP-activated protein kinase pathway. Hum Mol Genet (2007) 1.42
The GTPase activity and C-terminal cysteine of the Escherichia coli MnmE protein are essential for its tRNA modifying function. J Biol Chem (2003) 1.42
General aspects and neuropathology of X-linked adrenoleukodystrophy. Brain Pathol (2010) 1.41
Dopamine induces autophagic cell death and alpha-synuclein increase in human neuroblastoma SH-SY5Y cells. J Neurosci Res (2003) 1.41
The evolutionary origin of peroxisomes: an ER-peroxisome connection. Mol Biol Evol (2006) 1.39
[Study investigating the activity of several fluoroquinolones against Pseudomonas aeruginosa using the mutant prevention concentration]. Enferm Infecc Microbiol Clin (2006) 1.39
Expression of the biofilm-associated protein interferes with host protein receptors of Staphylococcus aureus and alters the infective process. Infect Immun (2002) 1.37
Phenotypic patterns of desminopathy associated with three novel mutations in the desmin gene. Neuromuscul Disord (2007) 1.35
Prognostic implication of clinical, radiologic, and pathologic features in patients with anaplastic gliomas. Cancer (2003) 1.33
Pro-NGF isolated from the human brain affected by Alzheimer's disease induces neuronal apoptosis mediated by p75NTR. Am J Pathol (2005) 1.33
Protein targets of oxidative damage in human neurodegenerative diseases with abnormal protein aggregates. Brain Pathol (2009) 1.33
Antioxidants halt axonal degeneration in a mouse model of X-adrenoleukodystrophy. Ann Neurol (2011) 1.31
Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy. Hum Mol Genet (2008) 1.27
Current and future pharmacological treatment strategies in X-linked adrenoleukodystrophy. Brain Pathol (2010) 1.27
PINK1-linked parkinsonism is associated with Lewy body pathology. Brain (2010) 1.27
Proteomic and oxidative stress analysis in human brain samples of Huntington disease. Free Radic Biol Med (2008) 1.27
PeroxisomeDB 2.0: an integrative view of the global peroxisomal metabolome. Nucleic Acids Res (2009) 1.24
Proteins in human brain cortex are modified by oxidation, glycoxidation, and lipoxidation. Effects of Alzheimer disease and identification of lipoxidation targets. J Biol Chem (2005) 1.24
Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy. Hum Mol Genet (2004) 1.23
Role of AMP-activated protein kinase in autophagy and proteasome function. Biochem Biophys Res Commun (2008) 1.22
Pathomechanisms underlying X-adrenoleukodystrophy: a three-hit hypothesis. Brain Pathol (2010) 1.20
A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity. PLoS Genet (2012) 1.20
Lafora bodies and neurological defects in malin-deficient mice correlate with impaired autophagy. Hum Mol Genet (2011) 1.16
Severe alterations in lipid composition of frontal cortex lipid rafts from Parkinson's disease and incidental Parkinson's disease. Mol Med (2011) 1.16
TAR DNA-Binding protein 43 accumulation in protein aggregate myopathies. J Neuropathol Exp Neurol (2009) 1.15
Evidence of oxidative stress in the neocortex in incidental Lewy body disease. J Neuropathol Exp Neurol (2005) 1.15
Role of proteasomes in the degradation of short-lived proteins in human fibroblasts under various growth conditions. Int J Biochem Cell Biol (2003) 1.13
MPP+ increases alpha-synuclein expression and ERK/MAP-kinase phosphorylation in human neuroblastoma SH-SY5Y cells. Brain Res (2002) 1.12
Intracellular protein degradation in mammalian cells: recent developments. Cell Mol Life Sci (2009) 1.11
PeroxisomeDB: a database for the peroxisomal proteome, functional genomics and disease. Nucleic Acids Res (2006) 1.08
A key role for the peroxisomal ABCD2 transporter in fatty acid homeostasis. Am J Physiol Endocrinol Metab (2008) 1.07
Amyloid-targeted therapeutics in Alzheimer's disease: use of human albumin in plasma exchange as a novel approach for Abeta mobilization. Drug News Perspect (2009) 1.07
Glucose induces autophagy under starvation conditions by a p38 MAPK-dependent pathway. Biochem J (2013) 1.06
Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage. Hum Mol Genet (2005) 1.06
DNA methylation of Alzheimer disease and tauopathy-related genes in postmortem brain. J Neuropathol Exp Neurol (2009) 1.05
A highly expressed miR-101 isomiR is a functional silencing small RNA. BMC Genomics (2013) 1.05
Transcriptomic profiling of TK2 deficient human skeletal muscle suggests a role for the p53 signalling pathway and identifies growth and differentiation factor-15 as a potential novel biomarker for mitochondrial myopathies. BMC Genomics (2014) 1.04
Lipid alterations in lipid rafts from Alzheimer's disease human brain cortex. J Alzheimers Dis (2010) 1.04
Up-regulation of the Kv3.4 potassium channel subunit in early stages of Alzheimer's disease. J Neurochem (2004) 1.04
Valproic acid induces antioxidant effects in X-linked adrenoleukodystrophy. Hum Mol Genet (2010) 1.03
A769662, a novel activator of AMP-activated protein kinase, inhibits non-proteolytic components of the 26S proteasome by an AMPK-independent mechanism. FEBS Lett (2008) 1.02
Withdrawal of essential amino acids increases autophagy by a pathway involving Ca2+/calmodulin-dependent kinase kinase-β (CaMKK-β). J Biol Chem (2012) 1.02
Clinical and myopathological evaluation of early- and late-onset subtypes of myofibrillar myopathy. Neuromuscul Disord (2011) 0.99
Oxidative stress underlying axonal degeneration in adrenoleukodystrophy: a paradigm for multifactorial neurodegenerative diseases? Biochim Biophys Acta (2012) 0.99
Molecular mechanisms of MLC1 and GLIALCAM mutations in megalencephalic leukoencephalopathy with subcortical cysts. Hum Mol Genet (2011) 0.98
Altered distribution of RhoA in Alzheimer's disease and AbetaPP overexpressing mice. J Alzheimers Dis (2010) 0.98
Increased 5-methylcytosine and decreased 5-hydroxymethylcytosine levels are associated with reduced striatal A2AR levels in Huntington's disease. Neuromolecular Med (2013) 0.98
Mouse liver PMP70 and ALDP: homomeric interactions prevail in vivo. Biochim Biophys Acta (2004) 0.98
Pro-NGF from Alzheimer's disease and normal human brain displays distinctive abilities to induce processing and nuclear translocation of intracellular domain of p75NTR and apoptosis. Am J Pathol (2006) 0.97
Dynamics of an F-actin aggresome generated by the actin-stabilizing toxin jasplakinolide. J Cell Sci (2008) 0.97
ABCD2 is abundant in adipose tissue and opposes the accumulation of dietary erucic acid (C22:1) in fat. J Lipid Res (2010) 0.96
Metabolomics of human brain aging and age-related neurodegenerative diseases. J Neuropathol Exp Neurol (2014) 0.96
Comparison of methods of DNA extraction for real-time PCR in a model of pleural tuberculosis. APMIS (2010) 0.96
Neuronal pentraxin 1 contributes to the neuronal damage evoked by amyloid-beta and is overexpressed in dystrophic neurites in Alzheimer's brain. J Neurosci (2006) 0.96
Inter-laboratory assessment of PrPSc typing in creutzfeldt-jakob disease: a Western blot study within the NeuroPrion Consortium. Brain Pathol (2008) 0.95
Neuropathology of Parkinson's disease with the R1441G mutation in LRRK2. Mov Disord (2009) 0.95
Characterization of human GTPBP3, a GTP-binding protein involved in mitochondrial tRNA modification. Mol Cell Biol (2008) 0.95
LRRK2 and neurodegeneration. Acta Neuropathol (2009) 0.95
Aquaporin expression in the cerebral cortex is increased at early stages of Alzheimer disease. Brain Res (2006) 0.95
Distinct patterns of APP processing in the CNS in autosomal-dominant and sporadic Alzheimer disease. Acta Neuropathol (2012) 0.95
A1 adenosine receptors accumulate in neurodegenerative structures in Alzheimer disease and mediate both amyloid precursor protein processing and tau phosphorylation and translocation. Brain Pathol (2003) 0.94
Risk of transmission of systemic transthyretin amyloidosis after domino liver transplantation. Liver Transpl (2010) 0.94
Protein oxidation in Huntington disease affects energy production and vitamin B6 metabolism. Free Radic Biol Med (2010) 0.94