OOIR: Observatory of International Research

Papers

(The TQCC of Acta Neuropathologica is 21. 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 2020-04-01 to 2024-04-01.)

Article	Citations
Neuropathology of COVID-19: a spectrum of vascular and acute disseminated encephalomyelitis (ADEM)-like pathology	383
Plasma p-tau231: a new biomarker for incipient Alzheimer’s disease pathology	282
The physiological roles of tau and Aβ: implications for Alzheimer’s disease pathology and therapeutics	219
Ultrasensitive RT-QuIC assay with high sensitivity and specificity for Lewy body-associated synucleinopathies	215
Distribution patterns of tau pathology in progressive supranuclear palsy	210
Plasma p-tau181 accurately predicts Alzheimer’s disease pathology at least 8 years prior to post-mortem and improves the clinical characterisation of cognitive decline	205
TREM2 activation on microglia promotes myelin debris clearance and remyelination in a model of multiple sclerosis	179
Distinct amyloid-β and tau-associated microglia profiles in Alzheimer’s disease	161
Cerebral blood flow decrease as an early pathological mechanism in Alzheimer's disease	151
The structural differences between patient-derived α-synuclein strains dictate characteristics of Parkinson’s disease, multiple system atrophy and dementia with Lewy bodies	145
Periphery and brain, innate and adaptive immunity in Parkinson’s disease	133
CDKN2A/B homozygous deletion is associated with early recurrence in meningiomas	116
APOE and TREM2 regulate amyloid-responsive microglia in Alzheimer’s disease	115
Correlates of critical illness-related encephalopathy predominate postmortem COVID-19 neuropathology	114
Accumulation of amyloid precursor protein C-terminal fragments triggers mitochondrial structure, function, and mitophagy defects in Alzheimer’s disease models and human brains	110
Neuropathological consensus criteria for the evaluation of Lewy pathology in post-mortem brains: a multi-centre study	103
Cryo-EM structures of tau filaments from Alzheimer’s disease with PET ligand APN-1607	96
The olfactory nerve is not a likely route to brain infection in COVID-19: a critical review of data from humans and animal models	92
Plasma biomarkers for Alzheimer’s Disease in relation to neuropathology and cognitive change	89
Microglia and monocytes in inflammatory CNS disease: integrating phenotype and function	83
Brain arteriolosclerosis	79
Diverse human astrocyte and microglial transcriptional responses to Alzheimer’s pathology	79
Tau strains shape disease	78
Microvascular injury and hypoxic damage: emerging neuropathological signatures in COVID-19	76
α-Synuclein in blood exosomes immunoprecipitated using neuronal and oligodendroglial markers distinguishes Parkinson’s disease from multiple system atrophy	75

Mitochondrial bioenergetic deficits in C9orf72 amyotrophic lateral sclerosis motor neurons cause dysfunctional axonal homeostasis	74
The mechanistic link between selective vulnerability of the locus coeruleus and neurodegeneration in Alzheimer’s disease	72
Patient-derived organoids and orthotopic xenografts of primary and recurrent gliomas represent relevant patient avatars for precision oncology	71
Lesion stage-dependent causes for impaired remyelination in MS	68
Frequency of LATE neuropathologic change across the spectrum of Alzheimer’s disease neuropathology: combined data from 13 community-based or population-based autopsy cohorts	67
Large-scale pathway specific polygenic risk and transcriptomic community network analysis identifies novel functional pathways in Parkinson disease	67
Analyzing microglial phenotypes across neuropathologies: a practical guide	65
Exosomes induce endolysosomal permeabilization as a gateway by which exosomal tau seeds escape into the cytosol	64
The subcellular arrangement of alpha-synuclein proteoforms in the Parkinson’s disease brain as revealed by multicolor STED microscopy	63
The role of hnRNPs in frontotemporal dementia and amyotrophic lateral sclerosis	60
Characterizing tau deposition in chronic traumatic encephalopathy (CTE): utility of the McKee CTE staging scheme	59
Inhibition of Bruton’s tyrosine kinase interferes with pathogenic B-cell development in inflammatory CNS demyelinating disease	56
TERT promoter mutation status is necessary and sufficient to diagnose IDH-wildtype diffuse astrocytic glioma with molecular features of glioblastoma	56
Neuron-specific activation of necroptosis signaling in multiple sclerosis cortical grey matter	53
Extrinsic immune cell-derived, but not intrinsic oligodendroglial factors contribute to oligodendroglial differentiation block in multiple sclerosis	53
Evidence of distinct α-synuclein strains underlying disease heterogeneity	53
Multiple system atrophy-associated oligodendroglial protein p25α stimulates formation of novel α-synuclein strain with enhanced neurodegenerative potential	53
Primary mismatch repair deficient IDH-mutant astrocytoma (PMMRDIA) is a distinct type with a poor prognosis	51
Physiological and pathological functions of TMEM106B: a gene associated with brain aging and multiple brain disorders	50
TDP-43 transports ribosomal protein mRNA to regulate axonal local translation in neuronal axons	50
Pediatric bithalamic gliomas have a distinct epigenetic signature and frequent EGFR exon 20 insertions resulting in potential sensitivity to targeted kinase inhibition	49
Frontal white matter lesions in Alzheimer’s disease are associated with both small vessel disease and AD-associated cortical pathology	47
Enhanced axonal response of mitochondria to demyelination offers neuroprotection: implications for multiple sclerosis	47
Perivascular space dilation is associated with vascular amyloid-β accumulation in the overlying cortex	47
White matter microglia heterogeneity in the CNS	47
Structure of Tau filaments in Prion protein amyloidoses	46
Atypical teratoid/rhabdoid tumors (ATRTs) with SMARCA4 mutation are molecularly distinct from SMARCB1-deficient cases	46
Interleukin-1 promotes autoimmune neuroinflammation by suppressing endothelial heme oxygenase-1 at the blood–brain barrier	45
Meningeal inflammation in multiple sclerosis induces phenotypic changes in cortical microglia that differentially associate with neurodegeneration	45
Patient-derived orthotopic xenografts of pediatric brain tumors: a St. Jude resource	45
SFPQ and Tau: critical factors contributing to rapid progression of Alzheimer’s disease	45
Infratentorial IDH-mutant astrocytoma is a distinct subtype	45
Biological sex and DNA repair deficiency drive Alzheimer’s disease via systemic metabolic remodeling and brain mitochondrial dysfunction	44
The coarse-grained plaque: a divergent Aβ plaque-type in early-onset Alzheimer’s disease	44
R1441G but not G2019S mutation enhances LRRK2 mediated Rab10 phosphorylation in human peripheral blood neutrophils	44
ETMR: a tumor entity in its infancy	43
Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS	43
Clinical and molecular heterogeneity of pineal parenchymal tumors: a consensus study	43
Insulin-like growth factor 2 (IGF2) protects against Huntington’s disease through the extracellular disposal of protein aggregates	42
Lewy pathology of the esophagus correlates with the progression of Lewy body disease: a Japanese cohort study of autopsy cases	42
Pyroptosis in Alzheimer’s disease: cell type-specific activation in microglia, astrocytes and neurons	40
Selective vulnerability of inhibitory networks in multiple sclerosis	39
C9orf72 loss-of-function: a trivial, stand-alone or additive mechanism in C9 ALS/FTD?	38
Complement component 3 from astrocytes mediates retinal ganglion cell loss during neuroinflammation	38
LATE-NC staging in routine neuropathologic diagnosis: an update	37
Profiling the neurovascular unit unveils detrimental effects of osteopontin on the blood–brain barrier in acute ischemic stroke	37
Genome-wide meta-analysis for Alzheimer’s disease cerebrospinal fluid biomarkers	37
PATZ1 fusions define a novel molecularly distinct neuroepithelial tumor entity with a broad histological spectrum	36
Neuronal activity modulates alpha-synuclein aggregation and spreading in organotypic brain slice cultures and in vivo	36
Supratentorial ependymoma in childhood: more than just RELA or YAP	36

Integrated molecular and clinical analysis of low-grade gliomas in children with neurofibromatosis type 1 (NF1)	36
Subgroup and subtype-specific outcomes in adult medulloblastoma	35
Maturation of neuronal AD-tau pathology involves site-specific phosphorylation of cytoplasmic and synaptic tau preceding conformational change and fibril formation	35
Making sense of missense variants in TTN-related congenital myopathies	34
Recurrent fusions in PLAGL1 define a distinct subset of pediatric-type supratentorial neuroepithelial tumors	34
The existence of Aβ strains and their potential for driving phenotypic heterogeneity in Alzheimer’s disease	34
Rainwater Charitable Foundation criteria for the neuropathologic diagnosis of progressive supranuclear palsy	34
Upstream open reading frame with NOTCH2NLC GGC expansion generates polyglycine aggregates and disrupts nucleocytoplasmic transport: implications for polyglycine diseases	34
Post-mortem analyses of PiB and flutemetamol in diffuse and cored amyloid-β plaques in Alzheimer’s disease	34
A multifactorial model of pathology for age of onset heterogeneity in familial Alzheimer’s disease	32
Frequent inactivating mutations of the PBAF complex gene PBRM1 in meningioma with papillary features	32
Cryo-EM structures of prion protein filaments from Gerstmann–Sträussler–Scheinker disease	32
Age-associated insolubility of parkin in human midbrain is linked to redox balance and sequestration of reactive dopamine metabolites	31
Non-IDH1-R132H IDH1/2 mutations are associated with increased DNA methylation and improved survival in astrocytomas, compared to IDH1-R132H mutations	31
Clear cell meningiomas are defined by a highly distinct DNA methylation profile and mutations in SMARCE1	31
Cross-platform transcriptional profiling identifies common and distinct molecular pathologies in Lewy body diseases	31
Seizure-mediated iron accumulation and dysregulated iron metabolism after status epilepticus and in temporal lobe epilepsy	31
CDKN2A deletion in supratentorial ependymoma with RELA alteration indicates a dismal prognosis: a retrospective analysis of the HIT ependymoma trial cohort	30
In vitro amplification of pathogenic tau conserves disease-specific bioactive characteristics	30
Distinct tau neuropathology and cellular profiles of an APOE3 Christchurch homozygote protected against autosomal dominant Alzheimer’s dementia	30
Altered ceramide metabolism is a feature in the extracellular vesicle-mediated spread of alpha-synuclein in Lewy body disorders	30
Lipids, lysosomes and mitochondria: insights into Lewy body formation from rare monogenic disorders	30
Complement-associated loss of CA2 inhibitory synapses in the demyelinated hippocampus impairs memory	30
Chronic traumatic encephalopathy (CTE): criteria for neuropathological diagnosis and relationship to repetitive head impacts	30
The proteome of granulovacuolar degeneration and neurofibrillary tangles in Alzheimer’s disease	29
BTK inhibition limits B-cell–T-cell interaction through modulation of B-cell metabolism: implications for multiple sclerosis therapy	29
Prion propagation estimated from brain diffusion MRI is subtype dependent in sporadic Creutzfeldt–Jakob disease	28
Multi-omic molecular profiling reveals potentially targetable abnormalities shared across multiple histologies of brain metastasis	28
Vesicle trafficking and lipid metabolism in synucleinopathy	28
Distinct characteristics of limbic-predominant age-related TDP-43 encephalopathy in Lewy body disease	28
Single-cell profiling of myasthenia gravis identifies a pathogenic T cell signature	27
Distinct clinicopathologic clusters of persons with TDP-43 proteinopathy	27
Mosaic trisomy of chromosome 1q in human brain tissue associates with unilateral polymicrogyria, very early-onset focal epilepsy, and severe developmental delay	26
Malignant transformation of a polymorphous low grade neuroepithelial tumor of the young (PLNTY)	26
Altered oligodendroglia and astroglia in chronic traumatic encephalopathy	26
Apolipoprotein E regulates lipid metabolism and α-synuclein pathology in human iPSC-derived cerebral organoids	25
Cryo-EM structures of amyloid-β filaments with the Arctic mutation (E22G) from human and mouse brains	25
CSF p-tau increase in response to Aβ-type and Danish-type cerebral amyloidosis and in the absence of neurofibrillary tangles	25
Accumulation of TMEM106B C-terminal fragments in neurodegenerative disease and aging	25
HIF-1α is involved in blood–brain barrier dysfunction and paracellular migration of bacteria in pneumococcal meningitis	24
Ultrastructural and biochemical classification of pathogenic tau, α-synuclein and TDP-43	24
HnRNP K mislocalisation is a novel protein pathology of frontotemporal lobar degeneration and ageing and leads to cryptic splicing	24
Epigenomic, genomic, and transcriptomic landscape of schwannomatosis	24
Glioblastomas with primitive neuronal component harbor a distinct methylation and copy-number profile with inactivation of TP53, PTEN, and RB1	23
APOE4 exacerbates α-synuclein seeding activity and contributes to neurotoxicity in Alzheimer’s disease with Lewy body pathology	23
Phenotypic diversity of genetic Creutzfeldt–Jakob disease: a histo-molecular-based classification	23
Reduction of advanced tau-mediated memory deficits by the MAP kinase p38γ	23
Tau immunotherapy is associated with glial responses in FTLD-tau	23
Impairment of the mitochondrial one-carbon metabolism enzyme SHMT2 causes a novel brain and heart developmental syndrome	23
MEF2 impairment underlies skeletal muscle atrophy in polyglutamine disease	23
Necrotic reshaping of the glioma microenvironment drives disease progression	23
Trafficking of the glutamate transporter is impaired in LRRK2-related Parkinson’s disease	23
Heterogeneity in α-synuclein fibril activity correlates to disease phenotypes in Lewy body dementia	23
CRISPR deletion of the C9ORF72 promoter in ALS/FTD patient motor neurons abolishes production of dipeptide repeat proteins and rescues neurodegeneration	22
Oligodendroglia heterogeneity in the human central nervous system	22
The oncogenic fusion landscape in pediatric CNS neoplasms	22
miR155 regulation of behavior, neuropathology, and cortical transcriptomics in Alzheimer's disease	22
Wolframin is a novel regulator of tau pathology and neurodegeneration	22
MOG-expressing teratoma followed by MOG-IgG-positive optic neuritis	21
Targeting cancer stem cells in medulloblastoma by inhibiting AMBRA1 dual function in autophagy and STAT3 signalling	21
Developmental malformations in Huntington disease: neuropathologic evidence of focal neuronal migration defects in a subset of adult brains	21
Retinal pathological features and proteome signatures of Alzheimer’s disease	21