OOIR: Observatory of International Research

Papers

(The TQCC of Acta Neuropathologica is 20. The table below lists those papers that are above that threshold based on CrossRef citation counts [max. 250 papers]. The publications cover those that have been published in the past four years, i.e., from 2020-09-01 to 2024-09-01.)

Article	Citations
Plasma p-tau231: a new biomarker for incipient Alzheimer’s disease pathology	330
Distinct amyloid-β and tau-associated microglia profiles in Alzheimer’s disease	191
Periphery and brain, innate and adaptive immunity in Parkinson’s disease	157
Accumulation of amyloid precursor protein C-terminal fragments triggers mitochondrial structure, function, and mitophagy defects in Alzheimer’s disease models and human brains	137
Neuropathological consensus criteria for the evaluation of Lewy pathology in post-mortem brains: a multi-centre study	128
Cryo-EM structures of tau filaments from Alzheimer’s disease with PET ligand APN-1607	118
Plasma biomarkers for Alzheimer’s Disease in relation to neuropathology and cognitive change	115
Microglia and monocytes in inflammatory CNS disease: integrating phenotype and function	108
The olfactory nerve is not a likely route to brain infection in COVID-19: a critical review of data from humans and animal models	99
Diverse human astrocyte and microglial transcriptional responses to Alzheimer’s pathology	98
Brain arteriolosclerosis	92
α-Synuclein in blood exosomes immunoprecipitated using neuronal and oligodendroglial markers distinguishes Parkinson’s disease from multiple system atrophy	90
Tau strains shape disease	88
Mitochondrial bioenergetic deficits in C9orf72 amyotrophic lateral sclerosis motor neurons cause dysfunctional axonal homeostasis	86
The mechanistic link between selective vulnerability of the locus coeruleus and neurodegeneration in Alzheimer’s disease	85
Patient-derived organoids and orthotopic xenografts of primary and recurrent gliomas represent relevant patient avatars for precision oncology	80
Frequency of LATE neuropathologic change across the spectrum of Alzheimer’s disease neuropathology: combined data from 13 community-based or population-based autopsy cohorts	79
The subcellular arrangement of alpha-synuclein proteoforms in the Parkinson’s disease brain as revealed by multicolor STED microscopy	78
Exosomes induce endolysosomal permeabilization as a gateway by which exosomal tau seeds escape into the cytosol	74
Analyzing microglial phenotypes across neuropathologies: a practical guide	73
Pyroptosis in Alzheimer’s disease: cell type-specific activation in microglia, astrocytes and neurons	71
Extrinsic immune cell-derived, but not intrinsic oligodendroglial factors contribute to oligodendroglial differentiation block in multiple sclerosis	67
TERT promoter mutation status is necessary and sufficient to diagnose IDH-wildtype diffuse astrocytic glioma with molecular features of glioblastoma	65
Perivascular space dilation is associated with vascular amyloid-β accumulation in the overlying cortex	62
Neuron-specific activation of necroptosis signaling in multiple sclerosis cortical grey matter	61

Primary mismatch repair deficient IDH-mutant astrocytoma (PMMRDIA) is a distinct type with a poor prognosis	59
Multiple system atrophy-associated oligodendroglial protein p25α stimulates formation of novel α-synuclein strain with enhanced neurodegenerative potential	59
LATE-NC staging in routine neuropathologic diagnosis: an update	58
Chronic traumatic encephalopathy (CTE): criteria for neuropathological diagnosis and relationship to repetitive head impacts	57
Physiological and pathological functions of TMEM106B: a gene associated with brain aging and multiple brain disorders	57
Atypical teratoid/rhabdoid tumors (ATRTs) with SMARCA4 mutation are molecularly distinct from SMARCB1-deficient cases	55
Meningeal inflammation in multiple sclerosis induces phenotypic changes in cortical microglia that differentially associate with neurodegeneration	54
The coarse-grained plaque: a divergent Aβ plaque-type in early-onset Alzheimer’s disease	54
Genome-wide meta-analysis for Alzheimer’s disease cerebrospinal fluid biomarkers	54
White matter microglia heterogeneity in the CNS	53
Lewy pathology of the esophagus correlates with the progression of Lewy body disease: a Japanese cohort study of autopsy cases	53
Frontal white matter lesions in Alzheimer’s disease are associated with both small vessel disease and AD-associated cortical pathology	52
Structure of Tau filaments in Prion protein amyloidoses	52
R1441G but not G2019S mutation enhances LRRK2 mediated Rab10 phosphorylation in human peripheral blood neutrophils	49
Profiling the neurovascular unit unveils detrimental effects of osteopontin on the blood–brain barrier in acute ischemic stroke	49
Complement component 3 from astrocytes mediates retinal ganglion cell loss during neuroinflammation	49
Rainwater Charitable Foundation criteria for the neuropathologic diagnosis of progressive supranuclear palsy	47
Clinical and molecular heterogeneity of pineal parenchymal tumors: a consensus study	47
Selective vulnerability of inhibitory networks in multiple sclerosis	46
C9orf72 loss-of-function: a trivial, stand-alone or additive mechanism in C9 ALS/FTD?	46
Retinal pathological features and proteome signatures of Alzheimer’s disease	44
Distinct tau neuropathology and cellular profiles of an APOE3 Christchurch homozygote protected against autosomal dominant Alzheimer’s dementia	43
Integrated molecular and clinical analysis of low-grade gliomas in children with neurofibromatosis type 1 (NF1)	42
Subgroup and subtype-specific outcomes in adult medulloblastoma	41
Neuronal activity modulates alpha-synuclein aggregation and spreading in organotypic brain slice cultures and in vivo	40
Supratentorial ependymoma in childhood: more than just RELA or YAP	39
Cryo-EM structures of prion protein filaments from Gerstmann–Sträussler–Scheinker disease	39
Recurrent fusions in PLAGL1 define a distinct subset of pediatric-type supratentorial neuroepithelial tumors	38
Maturation of neuronal AD-tau pathology involves site-specific phosphorylation of cytoplasmic and synaptic tau preceding conformational change and fibril formation	38
PATZ1 fusions define a novel molecularly distinct neuroepithelial tumor entity with a broad histological spectrum	38
Upstream open reading frame with NOTCH2NLC GGC expansion generates polyglycine aggregates and disrupts nucleocytoplasmic transport: implications for polyglycine diseases	38
BTK inhibition limits B-cell–T-cell interaction through modulation of B-cell metabolism: implications for multiple sclerosis therapy	37
Seizure-mediated iron accumulation and dysregulated iron metabolism after status epilepticus and in temporal lobe epilepsy	37
Cross-platform transcriptional profiling identifies common and distinct molecular pathologies in Lewy body diseases	37
Age-associated insolubility of parkin in human midbrain is linked to redox balance and sequestration of reactive dopamine metabolites	36
Making sense of missense variants in TTN-related congenital myopathies	36
In vitro amplification of pathogenic tau conserves disease-specific bioactive characteristics	35
Cryo-EM structures of amyloid-β filaments with the Arctic mutation (E22G) from human and mouse brains	35
A multifactorial model of pathology for age of onset heterogeneity in familial Alzheimer’s disease	34
Clear cell meningiomas are defined by a highly distinct DNA methylation profile and mutations in SMARCE1	34
Non-IDH1-R132H IDH1/2 mutations are associated with increased DNA methylation and improved survival in astrocytomas, compared to IDH1-R132H mutations	34
Complement-associated loss of CA2 inhibitory synapses in the demyelinated hippocampus impairs memory	34
Lipids, lysosomes and mitochondria: insights into Lewy body formation from rare monogenic disorders	33
Altered ceramide metabolism is a feature in the extracellular vesicle-mediated spread of alpha-synuclein in Lewy body disorders	33
Single-cell profiling of myasthenia gravis identifies a pathogenic T cell signature	32
The proteome of granulovacuolar degeneration and neurofibrillary tangles in Alzheimer’s disease	32
APOE4 exacerbates α-synuclein seeding activity and contributes to neurotoxicity in Alzheimer’s disease with Lewy body pathology	32
Ultrastructural and biochemical classification of pathogenic tau, α-synuclein and TDP-43	32
Accumulation of TMEM106B C-terminal fragments in neurodegenerative disease and aging	31
Distinct characteristics of limbic-predominant age-related TDP-43 encephalopathy in Lewy body disease	31

Necrotic reshaping of the glioma microenvironment drives disease progression	31
Physiological β-amyloid clearance by the liver and its therapeutic potential for Alzheimer’s disease	30
Malignant transformation of a polymorphous low grade neuroepithelial tumor of the young (PLNTY)	30
Mosaic trisomy of chromosome 1q in human brain tissue associates with unilateral polymicrogyria, very early-onset focal epilepsy, and severe developmental delay	30
Trafficking of the glutamate transporter is impaired in LRRK2-related Parkinson’s disease	30
Spatiotemporal characterization of cellular tau pathology in the human locus coeruleus–pericoerulear complex by three-dimensional imaging	29
Glioblastomas with primitive neuronal component harbor a distinct methylation and copy-number profile with inactivation of TP53, PTEN, and RB1	29
Altered oligodendroglia and astroglia in chronic traumatic encephalopathy	29
Apolipoprotein E regulates lipid metabolism and α-synuclein pathology in human iPSC-derived cerebral organoids	29
Multi-omic molecular profiling reveals potentially targetable abnormalities shared across multiple histologies of brain metastasis	29
Oligodendroglia heterogeneity in the human central nervous system	28
Heterogeneity in α-synuclein fibril activity correlates to disease phenotypes in Lewy body dementia	27
Tau immunotherapy is associated with glial responses in FTLD-tau	27
Targeting cancer stem cells in medulloblastoma by inhibiting AMBRA1 dual function in autophagy and STAT3 signalling	27
Transmission of cervid prions to humanized mice demonstrates the zoonotic potential of CWD	27
HnRNP K mislocalisation is a novel protein pathology of frontotemporal lobar degeneration and ageing and leads to cryptic splicing	27
Rapid-CNS2: rapid comprehensive adaptive nanopore-sequencing of CNS tumors, a proof-of-concept study	27
Heterogeneity of white matter astrocytes in the human brain	26
Skeletal muscle provides the immunological micro-milieu for specific plasma cells in anti-synthetase syndrome-associated myositis	26
Patterns of amygdala region pathology in LATE-NC: subtypes that differ with regard to TDP-43 histopathology, genetic risk factors, and comorbid pathologies	26
Phenotypic diversity of genetic Creutzfeldt–Jakob disease: a histo-molecular-based classification	26
CSF p-tau increase in response to Aβ-type and Danish-type cerebral amyloidosis and in the absence of neurofibrillary tangles	26
ApoE4 inhibition of VMAT2 in the locus coeruleus exacerbates Tau pathology in Alzheimer’s disease	26
FMNL2 regulates gliovascular interactions and is associated with vascular risk factors and cerebrovascular pathology in Alzheimer’s disease	25
Mutations within FGFR1 are associated with superior outcome in a series of 83 diffuse midline gliomas with H3F3A K27M mutations	25
Epigenomic, genomic, and transcriptomic landscape of schwannomatosis	25
Germline-driven replication repair-deficient high-grade gliomas exhibit unique hypomethylation patterns	25
Biomarkers for parkinsonian disorders in CNS-originating EVs: promise and challenges	25
The oncogenic fusion landscape in pediatric CNS neoplasms	25
Impairment of the mitochondrial one-carbon metabolism enzyme SHMT2 causes a novel brain and heart developmental syndrome	25
Developmental malformations in Huntington disease: neuropathologic evidence of focal neuronal migration defects in a subset of adult brains	25
Vagus nerve inflammation contributes to dysautonomia in COVID-19	24
TDP-43 drives synaptic and cognitive deterioration following traumatic brain injury	24
Wolframin is a novel regulator of tau pathology and neurodegeneration	24
Systemic sclerosis-associated myositis features minimal inflammation and characteristic capillary pathology	23
Intellectual disability: dendritic anomalies and emerging genetic perspectives	23
New SNCA mutation and structures of α-synuclein filaments from juvenile-onset synucleinopathy	23
Increased pyroptosis activation in white matter microglia is associated with neuronal loss in ALS motor cortex	23
Dominant-acting CSF1R variants cause microglial depletion and altered astrocytic phenotype in zebrafish and adult-onset leukodystrophy	23
MOG-expressing teratoma followed by MOG-IgG-positive optic neuritis	22
Alzheimer disease neuropathology in a patient previously treated with aducanumab	22
Association of small vessel disease with tau pathology	22
Association of probable REM sleep behavior disorder with pathology and years of contact sports play in chronic traumatic encephalopathy	22
A new non-aggregative splicing isoform of human Tau is decreased in Alzheimer’s disease	22
Oligosarcomas, IDH-mutant are distinct and aggressive	22
Transcriptomic analysis of frontotemporal lobar degeneration with TDP-43 pathology reveals cellular alterations across multiple brain regions	22
Galectin-3 is elevated in CSF and is associated with Aβ deposits and tau aggregates in brain tissue in Alzheimer’s disease	22
Limbic-predominant age-related TDP-43 encephalopathy neuropathological change (LATE-NC) is independently associated with dementia and strongly associated with arteriolosclerosis in the oldest-old	21
DDX17 is involved in DNA damage repair and modifies FUS toxicity in an RGG-domain dependent manner	21
TREM2 expression in the brain and biological fluids in prion diseases	21
EZHIP: a new piece of the puzzle towards understanding pediatric posterior fossa ependymoma	21
DNA methylation-based classification of malformations of cortical development in the human brain	21
Consequences of variability in α-synuclein fibril structure on strain biology	21
TERT promoter mutation and chromosome 6 loss define a high-risk subtype of ependymoma evolving from posterior fossa subependymoma	21
Genome-wide association study and functional validation implicates JADE1 in tauopathy	21
Alzheimer’s disease neuropathological change three decades after iatrogenic amyloid-β transmission	21
Adaptive structural changes in the motor cortex and white matter in Parkinson’s disease	21
Immunisation with UB-312 in the Thy1SNCA mouse prevents motor performance deficits and oligomeric α-synuclein accumulation in the brain and gut	20
Molecular characterization of CNS paragangliomas identifies cauda equina paragangliomas as a distinct tumor entity	20
Transmissible α-synuclein seeding activity in brain and stomach of patients with Parkinson’s disease	20
Neuronal spreading and plaque induction of intracellular Aβ and its disruption of Aβ homeostasis	20
Cross-regional homeostatic and reactive glial signatures in multiple sclerosis	20
Phosphorylated tau in the retina correlates with tau pathology in the brain in Alzheimer’s disease and primary tauopathies	20
TDP-43 interacts with pathological τ protein in Alzheimer’s disease	20