OOIR: Observatory of International Research

Papers

(The TQCC of Acta Neuropathologica is 35. 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 2022-06-01 to 2026-06-01.)

Article	Citations
A novel subtype of sporadic Creutzfeldt–Jakob disease with PRNP codon 129MM genotype and PrP plaques	222
Assessment of PABPN1 nuclear inclusions on a large cohort of patients and in a human xenograft model of oculopharyngeal muscular dystrophy	203
The proteomic landscape of glioblastoma recurrence reveals novel and targetable immunoregulatory drivers	145
Comprehensive assessment of TDP-43 neuropathology data in the National Alzheimer’s Coordinating Center database	133
Co-registration of MALDI-MSI and histology demonstrates gangliosides co-localize with amyloid beta plaques in Alzheimer’s disease	131
Macrophages and endothelial cells in the neurovascular unit	123
Regulated cell death and its role in Alzheimer’s disease and amyotrophic lateral sclerosis	104
Identification of high-performing antibodies for the reliable detection of Tau proteoforms by Western blotting and immunohistochemistry	89
Integrative multi-omics reveals two biologically distinct groups of pilocytic astrocytoma	87
RNA aptamer reveals nuclear TDP-43 pathology is an early aggregation event that coincides with STMN-2 cryptic splicing and precedes clinical manifestation in ALS	86
RNA methyltransferase NSun2 deficiency promotes neurodegeneration through epitranscriptomic regulation of tau phosphorylation	85
Metabologenomic characterization uncovers a clinically aggressive IDH mutant glioma subtype	83
A familial missense variant in the Alzheimer’s disease gene SORL1 impairs its maturation and endosomal sorting	81
Cryptic exon detection and transcriptomic changes revealed in single-nuclei RNA sequencing of C9ORF72 patients spanning the ALS-FTD spectrum	78
BTK inhibition limits microglia-perpetuated CNS inflammation and promotes myelin repair	75
TDP-43 pathology in the retina of patients with frontotemporal lobar degeneration	74
Even heterozygous loss of CDKN2A/B greatly accelerates recurrence in aggressive meningioma	67
The Alzheimer’s disease GWAS risk alleles in the ABCA7 promoter and 5’ region reduce ABCA7 expression	65
Detection of blood–brain barrier disruption in brains of patients with COVID-19, but no evidence of brain penetration by SARS-CoV-2	63
Cell-specific MAPT gene expression is preserved in neuronal and glial tau cytopathologies in progressive supranuclear palsy	63
Genetical and epigenetical profiling identifies two subgroups of pineal parenchymal tumors of intermediate differentiation (PPTID) with distinct molecular, histological and clinical characteristics	60
Biomarkers for parkinsonian disorders in CNS-originating EVs: promise and challenges	60
Multi-level profiling unravels mitochondrial dysfunction in myotonic dystrophy type 2	59
TREM2 gene expression associations with Alzheimer’s disease neuropathology are region-specific: implications for cortical versus subcortical microglia	58
Progranulin deficiency results in sex-dependent alterations in microglia in response to demyelination	57

Jorge Cervós-Navarro 1930–2021	56
Variants in DTNA cause a mild, dominantly inherited muscular dystrophy	56
Clinicopathological and molecular characterization of three cases classified by DNA-methylation profiling as “Glioneuronal Tumors, NOS, Subtype A”	54
From metabolomics to proteomics: understanding the role of dopa decarboxylase in Parkinson’s disease. Scientific commentary on: “Comprehensive proteomics of CSF, plasma, and urine identify DDC and oth	54
Clinical applicability of miR517a detection in liquid biopsies of ETMR patients	54
PolyGA targets the ER stress-adaptive response by impairing GRP75 function at the MAM in C9ORF72-ALS/FTD	53
Expanding the spectrum of amyloid-β plaque pathology: the Down syndrome associated ‘bird-nest plaque’	53
Landscape of brain myeloid cell transcriptome along the spatiotemporal progression of Alzheimer’s disease reveals distinct sequential responses to Aβ and tau	52
Myeloid cell iron uptake pathways and paramagnetic rim formation in multiple sclerosis	52
Correction to: MET receptor serves as a promising target in melanoma brain metastases	51
Spinal astrocyte dysfunction drives motor neuron loss in late-onset spinal muscular atrophy	51
Xenografted human iPSC-derived neurons with the familial Alzheimer’s disease APPV717I mutation reveal dysregulated transcriptome signatures linked to synaptic function and implicate LINGO2 as a diseas	51
Phosphatidylinositol-3,4,5-trisphosphate interacts with alpha-synuclein and initiates its aggregation and formation of Parkinson’s disease-related fibril polymorphism	51
A CHCHD6–APP axis connects amyloid and mitochondrial pathology in Alzheimer’s disease	50
Novel lissencephaly-associated NDEL1 variant reveals distinct roles of NDE1 and NDEL1 in nucleokinesis and human cortical malformations	48
Neuropathologic scales of cerebrovascular disease associated with diffusion changes on MRI	48
CSF p-tau205: a biomarker of tau pathology in Alzheimer’s disease	48
Myofiber-type-dependent ‘boulder’ or ‘multitudinous pebble’ formations across distinct amylopectinoses	47
The variance in phosphorylated, insoluble ⍺-synuclein in humans, rats, and mice is not mainly driven by biological sex	47
Physiological aging and inflammation-induced cellular senescence may contribute to oligodendroglial dysfunction in MS	46
Chronic traumatic encephalopathy in a female ex-professional Australian rules footballer	45
Independent prognostic impact of DNA methylation class and chromosome 1p loss in WHO grade 2 and 3 meningioma undergoing adjuvant high-dose radiotherapy: comprehensive molecular analysis of EORTC 2204	44
From methylation to myelination: epigenomic and transcriptomic profiling of chronic inactive demyelinated multiple sclerosis lesions	44
Comprehensive proteomics of CSF, plasma, and urine identify DDC and other biomarkers of early Parkinson’s disease	43
Clinically unfavorable transcriptome subtypes of non-WNT/non-SHH medulloblastomas are associated with a predominance in proliferating and progenitor-like cell subpopulations	43
Co-pathology may impact outcomes of amyloid-targeting treatments: clinicopathological results from two patients treated with aducanumab	43
Impaired GABAergic regulation and developmental immaturity in interneurons derived from the medial ganglionic eminence in the tuberous sclerosis complex	43
Multiplatform molecular analyses refine classification of gliomas arising in patients with neurofibromatosis type 1	43
Cortical-sparing chronic traumatic encephalopathy (CSCTE): a distinct subtype of CTE	43
Somatic mosaic SOX10 indel mutations underlie a form of segmental schwannomatosis	43
The prevalence and topography of spinal cord demyelination in multiple sclerosis: a retrospective study	42
Tau seeds occur before earliest Alzheimer’s changes and are prevalent across neurodegenerative diseases	42
Oncohistone interactome profiling uncovers contrasting oncogenic mechanisms and identifies potential therapeutic targets in high grade glioma	42
Regional AT-8 reactive tau species correlate with intracellular Aβ levels in cases of low AD neuropathologic change	41
p-tau Ser356 is associated with Alzheimer’s disease pathology and is lowered in brain slice cultures using the NUAK inhibitor WZ4003	41
Alteration of LARGE1 abundance in patients and a mouse model of 5q-associated spinal muscular atrophy	40
Decreased dystrophin expression and elevated dystrophin-targeting miRNAs in anti-HMGCR immune-mediated necrotizing myopathy	40
Concussion leads to widespread axonal sodium channel loss and disruption of the node of Ranvier	40
Disruption of the blood–brain barrier is correlated with spike endocytosis by ACE2 + endothelia in the CNS microvasculature in fatal COVID-19. Scientific commentary on "Detection of blood–brain barrie	39
Integrated genetic analyses of immunodeficiency-associated Epstein-Barr virus- (EBV) positive primary CNS lymphomas	38
Isoform-specific patterns of tau burden and neuronal degeneration in MAPT-associated frontotemporal lobar degeneration	38
Alteration of gene expression and protein solubility of the PI 5-phosphatase SHIP2 are correlated with Alzheimer’s disease pathology progression	38
Chronic traumatic encephalopathy (CTE): criteria for neuropathological diagnosis and relationship to repetitive head impacts	38
Single-cell DNA sequencing identifies risk-associated clonal complexity and evolutionary trajectories in childhood medulloblastoma development	38
An interaction between synapsin and C9orf72 regulates excitatory synapses and is impaired in ALS/FTD	38
Retinal pathological features and proteome signatures of Alzheimer’s disease	37
Urinary tract infections trigger synucleinopathy via the innate immune response	37
Expanded analysis of high-grade astrocytoma with piloid features identifies an epigenetically and clinically distinct subtype associated with neurofibromatosis type 1	37
Loss of p16 expression is a sensitive marker of CDKN2A homozygous deletion in malignant meningiomas	36
Integrative proteomics highlight presynaptic alterations and c-Jun misactivation as convergent pathomechanisms in ALS	36

More than meets the eye in Parkinson’s disease and other synucleinopathies: from proteinopathy to lipidopathy	36
Neuroinflammatory disease signatures in SPG11-related hereditary spastic paraplegia patients	35
Transmission experiments verify sporadic V2 prion in a patient with E200K mutation	35
Hemorrhagic lesion with detection of infected endothelial cells in human bornavirus encephalitis	35
“De novo replication repair deficient glioblastoma, IDH-wildtype” is a distinct glioblastoma subtype in adults that may benefit from immune checkpoint blockade	35
Characterisation of premature cell senescence in Alzheimer’s disease using single nuclear transcriptomics	35