Published in Biochem Biophys Res Commun on June 24, 2005
The cellular prion protein (PrP(C)): its physiological function and role in disease. Biochim Biophys Acta (2007) 2.09
The alpha-secretase-derived N-terminal product of cellular prion, N1, displays neuroprotective function in vitro and in vivo. J Biol Chem (2009) 1.27
Prion protein at the crossroads of physiology and disease. Trends Neurosci (2011) 1.26
The role of the octarepeat region in neuroprotective function of the cellular prion protein. Brain Pathol (2007) 1.25
Metal imaging in neurodegenerative diseases. Metallomics (2012) 0.93
Copper binding extrinsic to the octarepeat region in the prion protein. Curr Protein Pept Sci (2009) 0.91
Induction of ligand-specific PrP (C) signaling in human neuronal cells. Prion (2012) 0.87
Immunotherapy in prion disease. Nat Rev Neurol (2012) 0.86
Neuroprotective function of cellular prion protein in a mouse model of amyotrophic lateral sclerosis. Am J Pathol (2010) 0.86
Prion protein (PrP) gene-knockout cell lines: insight into functions of the PrP. Front Cell Dev Biol (2015) 0.85
A PrP(C)-caveolin-Lyn complex negatively controls neuronal GSK3β and serotonin 1B receptor. Sci Rep (2014) 0.83
Prion protein lacks robust cytoprotective activity in cultured cells. Mol Neurodegener (2008) 0.82
Endogenous prion protein attenuates experimentally induced colitis. Am J Pathol (2011) 0.82
Neuroprotective effects of the cellular prion protein in autoimmune optic neuritis. Am J Pathol (2011) 0.80
Prion protein scrapie and the normal cellular prion protein. Prion (2016) 0.75
Physiological Functions of the Cellular Prion Protein. Front Mol Biosci (2017) 0.75
Cellular Prion Protein (PrP(c)) and Hypoxia: True to Each Other in Good Times and in Bad, in Sickness, and in Health. Front Cell Neurosci (2016) 0.75
Functions of the cellular prion protein, the end of Moore's law, and Ockham's razor theory. Prion (2016) 0.75
Dual role of cellular prion protein in normal host and Alzheimer's disease. Proc Jpn Acad Ser B Phys Biol Sci (2017) 0.75
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol (2007) 6.58
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Science (2013) 6.34
Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Nat Genet (2010) 5.52
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions. Am J Pathol (2007) 5.02
A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain (2009) 4.40
Real-time imaging reveals the single steps of brain metastasis formation. Nat Med (2009) 3.95
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations. J Neuropathol Exp Neurol (2007) 2.97
Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies. J Neuropathol Exp Neurol (2008) 2.96
Tolerability of malaria chemoprophylaxis in non-immune travellers to sub-Saharan Africa: multicentre, randomised, double blind, four arm study. BMJ (2003) 2.87
Microglial Cx3cr1 knockout prevents neuron loss in a mouse model of Alzheimer's disease. Nat Neurosci (2010) 2.79
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies. Acta Neuropathol (2009) 2.76
Mutations in the colony stimulating factor 1 receptor (CSF1R) gene cause hereditary diffuse leukoencephalopathy with spheroids. Nat Genet (2011) 2.76
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Acta Neuropathol (2009) 2.68
Genetic prion disease: the EUROCJD experience. Hum Genet (2005) 2.64
Misfolded proteinase K-resistant hyperphosphorylated alpha-synuclein in aged transgenic mice with locomotor deterioration and in human alpha-synucleinopathies. J Clin Invest (2002) 2.47
Direct quantification of CSF alpha-synuclein by ELISA and first cross-sectional study in patients with neurodegeneration. Exp Neurol (2008) 2.31
hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations. Acta Neuropathol (2013) 2.27
The secreted beta-amyloid precursor protein ectodomain APPs alpha is sufficient to rescue the anatomical, behavioral, and electrophysiological abnormalities of APP-deficient mice. J Neurosci (2007) 2.25
ALDH1A1 is a marker of astrocytic differentiation during brain development and correlates with better survival in glioblastoma patients. Brain Pathol (2012) 2.14
Bevacizumab has differential and dose-dependent effects on glioma blood vessels and tumor cells. Clin Cancer Res (2011) 2.06
TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD. Acta Neuropathol (2008) 2.02
TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions. J Neuropathol Exp Neurol (2007) 2.02
Synapse formation and function is modulated by the amyloid precursor protein. J Neurosci (2006) 1.99
Hyperphosphorylation and insolubility of alpha-synuclein in transgenic mouse oligodendrocytes. EMBO Rep (2002) 1.91
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins. Acta Neuropathol (2013) 1.82
The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication. EMBO J (2011) 1.80
Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutations. Arch Neurol (2008) 1.78
FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations. Brain (2011) 1.76
Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol (2004) 1.74
Imaging glioma cell invasion in vivo reveals mechanisms of dissemination and peritumoral angiogenesis. Glia (2009) 1.69
Intratumoral homogeneity of MGMT promoter hypermethylation as demonstrated in serial stereotactic specimens from anaplastic astrocytomas and glioblastomas. Int J Cancer (2007) 1.67
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations. Acta Neuropathol (2013) 1.59
Dendritic pathology in prion disease starts at the synaptic spine. J Neurosci (2007) 1.57
A crucial role for matrix metalloproteinase 2 in osteocytic canalicular formation and bone metabolism. J Biol Chem (2006) 1.52
Identification of differentially expressed genes in scrapie-infected mouse brains by using global gene expression technology. J Virol (2004) 1.50
Effects of different experimental conditions on the PrPSc core generated by protease digestion: implications for strain typing and molecular classification of CJD. J Biol Chem (2004) 1.50
FET PET for the evaluation of untreated gliomas: correlation of FET uptake and uptake kinetics with tumour grading. Eur J Nucl Med Mol Imaging (2007) 1.47
Targeted disruption of the IA-2beta gene causes glucose intolerance and impairs insulin secretion but does not prevent the development of diabetes in NOD mice. Diabetes (2004) 1.43
Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J Biol Chem (2008) 1.40
Brain protein preservation largely depends on the postmortem storage temperature: implications for study of proteins in human neurologic diseases and management of brain banks: a BrainNet Europe Study. J Neuropathol Exp Neurol (2007) 1.38
Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD. Brain (2006) 1.35
Gamma-secretase inhibition reduces spine density in vivo via an amyloid precursor protein-dependent pathway. J Neurosci (2009) 1.35
Analysis of 18F-FET PET for grading of recurrent gliomas: is evaluation of uptake kinetics superior to standard methods? J Nucl Med (2006) 1.33
Alpha- and beta- cleavages of the amino-terminus of the cellular prion protein. Biol Cell (2004) 1.33
O-methylguanine-DNA methyltransferase (MGMT) mRNA expression predicts outcome in malignant glioma independent of MGMT promoter methylation. PLoS One (2011) 1.32
Selenium supplementation in patients with autoimmune thyroiditis decreases thyroid peroxidase antibodies concentrations. J Clin Endocrinol Metab (2002) 1.32
Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype. Neurobiol Aging (2012) 1.30
Calcium dysregulation in Alzheimer's disease. Neurochem Int (2007) 1.30
Low values of 5-hydroxymethylcytosine (5hmC), the "sixth base," are associated with anaplasia in human brain tumors. Int J Cancer (2012) 1.30
Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA. Acta Neuropathol (2012) 1.29
Cellular prion protein function in copper homeostasis and redox signalling at the synapse. J Neurochem (2003) 1.28
TDP-43 in cerebrospinal fluid of patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Arch Neurol (2008) 1.28
Expression of integrin alphavbeta3 in gliomas correlates with tumor grade and is not restricted to tumor vasculature. Brain Pathol (2008) 1.28
Imaging of integrin alpha(v)beta(3) expression in patients with malignant glioma by [18F] Galacto-RGD positron emission tomography. Neuro Oncol (2009) 1.27
Age-dependent cognitive decline and amygdala pathology in alpha-synuclein transgenic mice. Neurobiol Aging (2006) 1.26
Rescue of progranulin deficiency associated with frontotemporal lobar degeneration by alkalizing reagents and inhibition of vacuolar ATPase. J Neurosci (2011) 1.26
Distinct pathological subtypes of FTLD-FUS. Acta Neuropathol (2010) 1.26
The role of the octarepeat region in neuroprotective function of the cellular prion protein. Brain Pathol (2007) 1.25
Expression of FoxM1 is required for the proliferation of medulloblastoma cells and indicates worse survival of patients. Clin Cancer Res (2011) 1.23
Genetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B' region. J Biol Chem (2004) 1.23
Prion protein amino acid determinants of differential susceptibility and molecular feature of prion strains in mice and voles. PLoS Pathog (2008) 1.21
Pathological properties of the Parkinson's disease-associated protein DJ-1 in alpha-synucleinopathies and tauopathies: relevance for multiple system atrophy and Pick's disease. Acta Neuropathol (2004) 1.19
Generation of genuine prion infectivity by serial PMCA. Vet Microbiol (2007) 1.19
PrP gene polymorphisms in Cyprus goats and their association with resistance or susceptibility to natural scrapie. Vet J (2010) 1.19
MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain (2005) 1.18
Multiple events lead to dendritic spine loss in triple transgenic Alzheimer's disease mice. PLoS One (2010) 1.17
Conversion efficiency of bank vole prion protein in vitro is determined by residues 155 and 170, but does not correlate with the high susceptibility of bank voles to sheep scrapie in vivo. J Biol Chem (2006) 1.17
Clinical features and diagnosis of the MM2 cortical subtype of sporadic Creutzfeldt-Jakob disease. Arch Neurol (2006) 1.15
Positron emission tomography with O-(2-[18F]fluoroethyl)-l-tyrosine versus magnetic resonance imaging in the diagnosis of recurrent gliomas. Neurosurgery (2005) 1.15
Caprine prion gene polymorphisms are associated with decreased incidence of classical scrapie in goat herds in the United Kingdom. Vet Res (2011) 1.14
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration. Acta Neuropathol (2007) 1.14
A refined method for molecular typing reveals that co-occurrence of PrP(Sc) types in Creutzfeldt-Jakob disease is not the rule. Lab Invest (2007) 1.12
oxLDL uptake by dendritic cells induces upregulation of scavenger-receptors, maturation and differentiation. Atherosclerosis (2009) 1.11
Design of anti- and pro-aggregation variants to assess the effects of methionine oxidation in human prion protein. Proc Natl Acad Sci U S A (2009) 1.10
Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis. Acta Neuropathol (2014) 1.10
5-Aminolevulinic acid-induced protoporphyrin IX levels in tissue of human malignant brain tumors. Photochem Photobiol (2010) 1.10
A congenital mutation of the novel gene LRRC8 causes agammaglobulinemia in humans. J Clin Invest (2003) 1.09
Novel molecular stereotactic biopsy procedures reveal intratumoral homogeneity of loss of heterozygosity of 1p/19q and TP53 mutations in World Health Organization grade II gliomas. J Neuropathol Exp Neurol (2009) 1.09
Creutzfeldt-Jakob disease in a patient with an R208H mutation of the prion protein gene (PRNP) and a 17-kDa prion protein fragment. Acta Neuropathol (2005) 1.09
Presence of pluripotent CD133+ cells correlates with malignancy of gliomas. Mol Cell Neurosci (2008) 1.08
Methods for studying synaptosomal copper release. J Neurosci Methods (2003) 1.08
Cellular phenotyping of secretory and nuclear prion proteins associated with inherited prion diseases. J Biol Chem (2001) 1.08
Transcriptome analysis reveals altered cholesterol metabolism during the neurodegeneration in mouse scrapie model. J Neurochem (2007) 1.07
Brain-derived proteins in the CSF: do they correlate with brain pathology in CJD? BMC Neurol (2006) 1.06
Loss of glycosylation associated with the T183A mutation in human prion disease. Acta Neuropathol (2004) 1.06