Published in Acta Neuropathol Commun on February 28, 2014
X-ray fluorescence imaging reveals subcellular biometal disturbances in a childhood neurodegenerative disorder. Chem Sci (2014) 0.80
Restoration of intestinal function in an MPTP model of Parkinson's Disease. Sci Rep (2016) 0.75
Identification of Cerebral Metal Ion Imbalance in the Brain of Aging Octodon degus. Front Aging Neurosci (2017) 0.75
Coherent and Contradictory Facts, Feats and Fictions Associated with Metal Accumulation in Parkinson's Disease: Epicenter or Outcome, Yet a Demigod Question. Mol Neurobiol (2016) 0.75
Glucocerebrosidase mutations in clinical and pathologically proven Parkinson's disease. Brain (2009) 4.01
Hereditary parkinsonism with dementia is caused by mutations in ATP13A2, encoding a lysosomal type 5 P-type ATPase. Nat Genet (2006) 4.01
Isolation of a candidate gene for Menkes disease that encodes a potential heavy metal binding protein. Nat Genet (1993) 3.63
Kufs disease, the major adult form of neuronal ceroid lipofuscinosis, caused by mutations in CLN6. Am J Hum Genet (2011) 2.49
Loss of P-type ATPase ATP13A2/PARK9 function induces general lysosomal deficiency and leads to Parkinson disease neurodegeneration. Proc Natl Acad Sci U S A (2012) 2.16
Lysosomal acidification mechanisms. Annu Rev Physiol (2012) 2.15
Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses. Hum Mutat (2011) 2.13
The neuronal ceroid-lipofuscinoses: from past to present. Biochim Biophys Acta (2006) 1.88
Lipid storage disorders block lysosomal trafficking by inhibiting a TRP channel and lysosomal calcium release. Nat Commun (2012) 1.85
Iron, zinc and copper in the Alzheimer's disease brain: a quantitative meta-analysis. Some insight on the influence of citation bias on scientific opinion. Prog Neurobiol (2011) 1.80
Cognitive loss in zinc transporter-3 knock-out mice: a phenocopy for the synaptic and memory deficits of Alzheimer's disease? J Neurosci (2010) 1.74
Mutations in SLC30A10 cause parkinsonism and dystonia with hypermanganesemia, polycythemia, and chronic liver disease. Am J Hum Genet (2012) 1.71
Measuring steady-state and dynamic endoplasmic reticulum and Golgi Zn2+ with genetically encoded sensors. Proc Natl Acad Sci U S A (2011) 1.68
Neuronal ceroid lipofuscinosis in Merino sheep. Aust Vet J (2002) 1.53
Brain alpha-synuclein accumulation in multiple system atrophy, Parkinson's disease and progressive supranuclear palsy: a comparative investigation. Brain (2009) 1.44
Mutation of the parkinsonism gene ATP13A2 causes neuronal ceroid-lipofuscinosis. Hum Mol Genet (2012) 1.41
Zip14 is a complex broad-scope metal-ion transporter whose functional properties support roles in the cellular uptake of zinc and nontransferrin-bound iron. Am J Physiol Cell Physiol (2011) 1.32
Increasing Cu bioavailability inhibits Abeta oligomers and tau phosphorylation. Proc Natl Acad Sci U S A (2009) 1.29
ZIP8 is an iron and zinc transporter whose cell-surface expression is up-regulated by cellular iron loading. J Biol Chem (2012) 1.29
miR-7 and miR-214 are specifically expressed during neuroblastoma differentiation, cortical development and embryonic stem cells differentiation, and control neurite outgrowth in vitro. Biochem Biophys Res Commun (2010) 1.29
Tyrosine gated electron transfer is key to the toxic mechanism of Alzheimer's disease beta-amyloid. FASEB J (2004) 1.29
Developmental neurotoxicity testing in vitro: models for assessing chemical effects on neurite outgrowth. Neurotoxicology (2008) 1.28
Structure-function analysis of HKE4, a member of the new LIV-1 subfamily of zinc transporters. Biochem J (2004) 1.28
Assessment of chemical effects on neurite outgrowth in PC12 cells using high content screening. Toxicol Sci (2008) 1.27
Lysine 43 is trimethylated in subunit C from bovine mitochondrial ATP synthase and in storage bodies associated with batten disease. J Biol Chem (2004) 1.23
Protein kinase CK2 triggers cytosolic zinc signaling pathways by phosphorylation of zinc channel ZIP7. Sci Signal (2012) 1.22
Neuronal ceroid lipofuscinosis (nclf), a new disorder of the mouse linked to chromosome 9. Am J Med Genet (1998) 1.21
Insights into Zn2+ homeostasis in neurons from experimental and modeling studies. Am J Physiol Cell Physiol (2008) 1.20
A truncating mutation in ATP13A2 is responsible for adult-onset neuronal ceroid lipofuscinosis in Tibetan terriers. Neurobiol Dis (2011) 1.19
Zinc transporters, ZnT5 and ZnT7, are required for the activation of alkaline phosphatases, zinc-requiring enzymes that are glycosylphosphatidylinositol-anchored to the cytoplasmic membrane. J Biol Chem (2004) 1.18
Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6). Neurobiol Dis (2005) 1.14
A missense mutation (c.184C>T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Merino sheep whereas affected South Hampshire sheep have reduced levels of CLN6 mRNA. Biochim Biophys Acta (2006) 1.13
Picomolar concentrations of free zinc(II) ions regulate receptor protein-tyrosine phosphatase β activity. J Biol Chem (2012) 1.12
Selective intracellular release of copper and zinc ions from bis(thiosemicarbazonato) complexes reduces levels of Alzheimer disease amyloid-beta peptide. J Biol Chem (2007) 1.08
Cloning the Wilson disease gene. Nat Genet (1993) 1.04
Defective endoplasmic reticulum-resident membrane protein CLN6 affects lysosomal degradation of endocytosed arylsulfatase A. J Biol Chem (2004) 1.04
Metal ionophore treatment restores dendritic spine density and synaptic protein levels in a mouse model of Alzheimer's disease. PLoS One (2011) 1.02
A one base pair deletion in the canine ATP13A2 gene causes exon skipping and late-onset neuronal ceroid lipofuscinosis in the Tibetan terrier. PLoS Genet (2011) 1.02
A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571+1G>A) leading to excision of exon 3. Neurobiol Dis (2007) 1.02
Atp13a2-deficient mice exhibit neuronal ceroid lipofuscinosis, limited α-synuclein accumulation and age-dependent sensorimotor deficits. Hum Mol Genet (2013) 0.99
Diacetylbis(N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model. J Biol Chem (2011) 0.96
The hypoxia imaging agent CuII(atsm) is neuroprotective and improves motor and cognitive functions in multiple animal models of Parkinson's disease. J Exp Med (2012) 0.96
Zn²⁺ dyshomeostasis caused by loss of ATP13A2/PARK9 leads to lysosomal dysfunction and alpha-synuclein accumulation. Hum Mol Genet (2013) 0.96
Alterations of zinc transporter proteins ZnT-1, ZnT-4 and ZnT-6 in preclinical Alzheimer's disease brain. Brain Pathol (2009) 0.94
Metal ion physiopathology in neurodegenerative disorders. Neuromolecular Med (2009) 0.93
Effect of dietary iron deficiency and overload on the expression of ZIP metal-ion transporters in rat liver. Biometals (2011) 0.93
Presenilins promote the cellular uptake of copper and zinc and maintain copper chaperone of SOD1-dependent copper/zinc superoxide dismutase activity. J Biol Chem (2011) 0.92
Protein trafficking abnormalities in Drosophila tissues with impaired activity of the ZIP7 zinc transporter Catsup. Development (2013) 0.92
Activation of non-neuronal cells within the prenatal developing brain of sheep with neuronal ceroid lipofuscinosis. Brain Pathol (2006) 0.92
Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis. PLoS One (2012) 0.91
Manganese exposure induces α-synuclein aggregation in the frontal cortex of non-human primates. Toxicol Lett (2012) 0.91
Alterations in zinc transporter protein-1 (ZnT-1) in the brain of subjects with mild cognitive impairment, early, and late-stage Alzheimer's disease. Neurotox Res (2005) 0.91
The cation selectivity of the ZIP transporters. Curr Top Membr (2012) 0.90
The human ZIP4 transporter has two distinct binding affinities and mediates transport of multiple transition metals. Biochemistry (2012) 0.89
TRPML: transporters of metals in lysosomes essential for cell survival? Cell Calcium (2011) 0.88
Neuronal ceroid lipofuscinosis in Australian Merino sheep: a new animal model. Eur J Paediatr Neurol (2001) 0.88
Glutathione peroxidase 1 and glutathione are required to protect mouse astrocytes from iron-mediated hydrogen peroxide toxicity. J Neurosci Res (2006) 0.86
Inhibition of TDP-43 accumulation by bis(thiosemicarbazonato)-copper complexes. PLoS One (2012) 0.84
Zinc induces cell death in immortalized embryonic hippocampal cells via activation of Akt-GSK-3beta signaling. Exp Cell Res (2006) 0.84
Slc39a7/zip7 plays a critical role in development and zinc homeostasis in zebrafish. PLoS One (2012) 0.84
Increased zinc and manganese in parallel with neurodegeneration, synaptic protein changes and activation of Akt/GSK3 signaling in ovine CLN6 neuronal ceroid lipofuscinosis. PLoS One (2013) 0.82
High precision isotope measurements reveal poor control of copper metabolism in parkinsonism. Metallomics (2013) 0.79
Metallothioneins as dynamic markers for brain disease in lysosomal disorders. Ann Neurol (2014) 0.78
Induction of metallothionein synthesis in cultured human trophoblasts by cadmium and zinc. J Toxicol Environ Health (1984) 0.78
A novel zinc bis(thiosemicarbazone) complex for live cell imaging. J Biol Inorg Chem (2011) 0.77
Effects of a copper-deficient diet on the biochemistry, neural morphology and behavior of aged mice. PLoS One (2012) 0.76
The genome sequence of taurine cattle: a window to ruminant biology and evolution. Science (2009) 8.23
Metal-protein attenuation with iodochlorhydroxyquin (clioquinol) targeting Abeta amyloid deposition and toxicity in Alzheimer disease: a pilot phase 2 clinical trial. Arch Neurol (2003) 3.58
Tau deficiency induces parkinsonism with dementia by impairing APP-mediated iron export. Nat Med (2012) 3.31
Quantitative trait loci mapping in dairy cattle: review and meta-analysis. Genet Sel Evol (2004) 2.38
Rapid restoration of cognition in Alzheimer's transgenic mice with 8-hydroxy quinoline analogs is associated with decreased interstitial Abeta. Neuron (2008) 2.30
Improving outcome after stroke: overcoming the translational roadblock. Cerebrovasc Dis (2008) 2.26
An unusual dimeric structure of a Cu(I) bis(thiosemicarbazone) complex: implications for the mechanism of hypoxic selectivity of the Cu(II) derivatives. J Am Chem Soc (2002) 2.22
Bone-marrow-derived cells contribute to the recruitment of microglial cells in response to beta-amyloid deposition in APP/PS1 double transgenic Alzheimer mice. Neurobiol Dis (2005) 2.16
Hypoxia-inducible factor prolyl 4-hydroxylase inhibition. A target for neuroprotection in the central nervous system. J Biol Chem (2005) 1.95
Stroke research priorities for the next decade--A representative view of the European scientific community. Cerebrovasc Dis (2006) 1.75
Hypoxia induces microRNA miR-210 in vitro and in vivo ephrin-A3 and neuronal pentraxin 1 are potentially regulated by miR-210. FEBS Lett (2008) 1.75
Overexpression of Alzheimer's disease amyloid-beta opposes the age-dependent elevations of brain copper and iron. J Biol Chem (2002) 1.74
Minocycline prevents neurotoxicity induced by cerebrospinal fluid from patients with motor neurone disease. Brain (2002) 1.71
Nuclear factor-kappaB contributes to infarction after permanent focal ischemia. Stroke (2004) 1.58
Mitochondrial oxidative stress causes hyperphosphorylation of tau. PLoS One (2007) 1.55
Plasma amyloid beta42 and amyloid beta40 levels are associated with early cognitive dysfunction after cardiac surgery. Ann Thorac Surg (2009) 1.50
Protein disulfide isomerase in ALS mouse glia links protein misfolding with NADPH oxidase-catalyzed superoxide production. Hum Mol Genet (2012) 1.49
Role of p38 and p44/42 mitogen-activated protein kinases in microglia. Glia (2002) 1.49
Decreased serum zinc is an effect of ageing and not Alzheimer's disease. Metallomics (2014) 1.44
Antigenic differences and conservation among placental Plasmodium falciparum-infected erythrocytes and acquisition of variant-specific and cross-reactive antibodies. J Infect Dis (2006) 1.34
Angiotensin II-dependent TGF-β signaling contributes to Loeys-Dietz syndrome vascular pathogenesis. J Clin Invest (2013) 1.32
Nrf2 regulates antioxidant gene expression evoked by oxidized phospholipids in endothelial cells and murine arteries in vivo. Circ Res (2008) 1.32
Transcribed var genes associated with placental malaria in Malawian women. Infect Immun (2006) 1.32
Intrahippocampal injection of a lentiviral vector expressing Nrf2 improves spatial learning in a mouse model of Alzheimer's disease. Proc Natl Acad Sci U S A (2009) 1.31
Degradation of the Alzheimer disease amyloid beta-peptide by metal-dependent up-regulation of metalloprotease activity. J Biol Chem (2006) 1.29
Increasing Cu bioavailability inhibits Abeta oligomers and tau phosphorylation. Proc Natl Acad Sci U S A (2009) 1.29
Stable RNA interference: comparison of U6 and H1 promoters in endothelial cells and in mouse brain. J Gene Med (2006) 1.29
Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates. Neurochem Int (2012) 1.28
Pyrrolidine dithiocarbamate activates Akt and improves spatial learning in APP/PS1 mice without affecting beta-amyloid burden. J Neurosci (2007) 1.28
Transfer of copper between bis(thiosemicarbazone) ligands and intracellular copper-binding proteins. insights into mechanisms of copper uptake and hypoxia selectivity. Inorg Chem (2008) 1.26
Interactions between Alzheimer's disease and cerebral ischemia--focus on inflammation. Brain Res Brain Res Rev (2005) 1.26
Copper complexes of bis(thiosemicarbazones): from chemotherapeutics to diagnostic and therapeutic radiopharmaceuticals. Chem Soc Rev (2011) 1.24
Transplanted astrocytes internalize deposited beta-amyloid peptides in a transgenic mouse model of Alzheimer's disease. Glia (2008) 1.23
Insights into Zn2+ homeostasis in neurons from experimental and modeling studies. Am J Physiol Cell Physiol (2008) 1.20
Human intravenous immunoglobulin provides protection against Aβ toxicity by multiple mechanisms in a mouse model of Alzheimer's disease. J Neuroinflammation (2010) 1.18
Comparison of (64)Cu-complexing bifunctional chelators for radioimmunoconjugation: labeling efficiency, specific activity, and in vitro/in vivo stability. Bioconjug Chem (2012) 1.17
Neuronal zinc exchange with the blood vessel wall promotes cerebral amyloid angiopathy in an animal model of Alzheimer's disease. J Neurosci (2004) 1.15
Minocycline protects against permanent cerebral ischemia in wild type but not in matrix metalloprotease-9-deficient mice. J Cereb Blood Flow Metab (2005) 1.14
Neurotoxic, redox-competent Alzheimer's beta-amyloid is released from lipid membrane by methionine oxidation. J Biol Chem (2003) 1.14
Functionalized bis(thiosemicarbazonato) complexes of zinc and copper: synthetic platforms toward site-specific radiopharmaceuticals. Inorg Chem (2007) 1.13
Bulldog dwarfism in Dexter cattle is caused by mutations in ACAN. Mamm Genome (2007) 1.11
Nuclear factor erythroid 2-related factor 2 protects against beta amyloid. Mol Cell Neurosci (2008) 1.11
Pyrrolidine dithiocarbamate inhibits translocation of nuclear factor kappa-B in neurons and protects against brain ischaemia with a wide therapeutic time window. J Neurochem (2004) 1.10
Selective intracellular release of copper and zinc ions from bis(thiosemicarbazonato) complexes reduces levels of Alzheimer disease amyloid-beta peptide. J Biol Chem (2007) 1.08
Mechanisms of copper ion mediated Huntington's disease progression. PLoS One (2007) 1.08
Evidence for the toxicity of bidirectional transcripts and mitochondrial dysfunction in blood associated with small CGG expansions in the FMR1 gene in patients with parkinsonism. Genet Med (2011) 1.06
Deleterious role of superoxide dismutase in the mitochondrial intermembrane space. J Biol Chem (2008) 1.06
The Alzheimer's therapeutic PBT2 promotes amyloid-β degradation and GSK3 phosphorylation via a metal chaperone activity. J Neurochem (2011) 1.06
Congenital syndactyly in cattle: four novel mutations in the low density lipoprotein receptor-related protein 4 gene (LRP4). BMC Genet (2007) 1.06
The role and therapeutic potential of monocytic cells in Alzheimer's disease. Glia (2010) 1.04
Beta-amyloid precursor protein transgenic mice that harbor diffuse A beta deposits but do not form plaques show increased ischemic vulnerability: role of inflammation. Proc Natl Acad Sci U S A (2002) 1.04
Zinc and copper modulate Alzheimer Abeta levels in human cerebrospinal fluid. Neurobiol Aging (2007) 1.04
Gender and genetic background effects on brain metal levels in APP transgenic and normal mice: implications for Alzheimer beta-amyloid pathology. J Inorg Biochem (2006) 1.02
Metal ionophore treatment restores dendritic spine density and synaptic protein levels in a mouse model of Alzheimer's disease. PLoS One (2011) 1.02
A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571+1G>A) leading to excision of exon 3. Neurobiol Dis (2007) 1.02
C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress. Mol Neurodegener (2011) 1.01
Radioiodinated clioquinol as a biomarker for beta-amyloid: Zn complexes in Alzheimer's disease. Aging Cell (2006) 1.01
Elevated labile Cu is associated with oxidative pathology in Alzheimer disease. Free Radic Biol Med (2011) 1.01
Cationic, linear Au(I) N-heterocyclic carbene complexes: synthesis, structure and anti-mitochondrial activity. Dalton Trans (2006) 1.00
Effects of human intravenous immunoglobulin on amyloid pathology and neuroinflammation in a mouse model of Alzheimer's disease. J Neuroinflammation (2012) 0.99
Oxidative modification of proteins in the frontal cortex of Alzheimer's disease brain. Neurobiol Aging (2005) 0.99
Distal occlusion of the middle cerebral artery in mice: are we ready to assess long-term functional outcome? Transl Stroke Res (2013) 0.98
Acetylacetonate bis(thiosemicarbazone) complexes of copper and nickel: towards new copper radiopharmaceuticals. Dalton Trans (2004) 0.98
ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation. PLoS One (2013) 0.97
The microglia-activating potential of thrombin: the protease is not involved in the induction of proinflammatory cytokines and chemokines. J Biol Chem (2004) 0.97
Diacetylbis(N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model. J Biol Chem (2011) 0.96
The hypoxia imaging agent CuII(atsm) is neuroprotective and improves motor and cognitive functions in multiple animal models of Parkinson's disease. J Exp Med (2012) 0.96
Granulocyte colony stimulating factor attenuates inflammation in a mouse model of amyotrophic lateral sclerosis. J Neuroinflammation (2011) 0.96
An impaired mitochondrial electron transport chain increases retention of the hypoxia imaging agent diacetylbis(4-methylthiosemicarbazonato)copperII. Proc Natl Acad Sci U S A (2011) 0.96
A domain level interaction network of amyloid precursor protein and Abeta of Alzheimer's disease. Proteomics (2010) 0.96
Use of model organisms for the study of neuronal ceroid lipofuscinosis. Biochim Biophys Acta (2013) 0.96
Linkage disequilibrium on chromosome 6 in Australian Holstein-Friesian cattle. Genet Sel Evol (2006) 0.96
Multiple cellular and molecular mechanisms are involved in human Aβ clearance by transplanted adult astrocytes. Glia (2011) 0.96
Proteomic analysis of protein oxidation in Alzheimer's disease brain. Electrophoresis (2002) 0.95
Heritability and phenotypic variation of canine hip dysplasia radiographic traits in a cohort of Australian German shepherd dogs. PLoS One (2012) 0.94
Glycosylation changes in Alzheimer's disease as revealed by a proteomic approach. Neurosci Lett (2004) 0.94
beta-Amyloid infusion results in delayed and age-dependent learning deficits without role of inflammation or beta-amyloid deposits. Proc Natl Acad Sci U S A (2006) 0.94
Iron accumulates in Huntington's disease neurons: protection by deferoxamine. PLoS One (2013) 0.93
Platinum-based inhibitors of amyloid-beta as therapeutic agents for Alzheimer's disease. Proc Natl Acad Sci U S A (2008) 0.93