Acta Neuropathologica

(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.)
Neuropathology of COVID-19: a spectrum of vascular and acute disseminated encephalomyelitis (ADEM)-like pathology383
Plasma p-tau231: a new biomarker for incipient Alzheimer’s disease pathology282
The physiological roles of tau and Aβ: implications for Alzheimer’s disease pathology and therapeutics219
Ultrasensitive RT-QuIC assay with high sensitivity and specificity for Lewy body-associated synucleinopathies215
Distribution patterns of tau pathology in progressive supranuclear palsy210
Plasma p-tau181 accurately predicts Alzheimer’s disease pathology at least 8 years prior to post-mortem and improves the clinical characterisation of cognitive decline205
TREM2 activation on microglia promotes myelin debris clearance and remyelination in a model of multiple sclerosis179
Distinct amyloid-β and tau-associated microglia profiles in Alzheimer’s disease161
Cerebral blood flow decrease as an early pathological mechanism in Alzheimer's disease151
The structural differences between patient-derived α-synuclein strains dictate characteristics of Parkinson’s disease, multiple system atrophy and dementia with Lewy bodies145
Periphery and brain, innate and adaptive immunity in Parkinson’s disease133
CDKN2A/B homozygous deletion is associated with early recurrence in meningiomas116
APOE and TREM2 regulate amyloid-responsive microglia in Alzheimer’s disease115
Correlates of critical illness-related encephalopathy predominate postmortem COVID-19 neuropathology114
Accumulation of amyloid precursor protein C-terminal fragments triggers mitochondrial structure, function, and mitophagy defects in Alzheimer’s disease models and human brains110
Neuropathological consensus criteria for the evaluation of Lewy pathology in post-mortem brains: a multi-centre study103
Cryo-EM structures of tau filaments from Alzheimer’s disease with PET ligand APN-160796
The olfactory nerve is not a likely route to brain infection in COVID-19: a critical review of data from humans and animal models92
Plasma biomarkers for Alzheimer’s Disease in relation to neuropathology and cognitive change89
Microglia and monocytes in inflammatory CNS disease: integrating phenotype and function83
Brain arteriolosclerosis79
Diverse human astrocyte and microglial transcriptional responses to Alzheimer’s pathology79
Tau strains shape disease78
Microvascular injury and hypoxic damage: emerging neuropathological signatures in COVID-1976
α-Synuclein in blood exosomes immunoprecipitated using neuronal and oligodendroglial markers distinguishes Parkinson’s disease from multiple system atrophy75
Mitochondrial bioenergetic deficits in C9orf72 amyotrophic lateral sclerosis motor neurons cause dysfunctional axonal homeostasis74
The mechanistic link between selective vulnerability of the locus coeruleus and neurodegeneration in Alzheimer’s disease72
Patient-derived organoids and orthotopic xenografts of primary and recurrent gliomas represent relevant patient avatars for precision oncology71
Lesion stage-dependent causes for impaired remyelination in MS68
Frequency of LATE neuropathologic change across the spectrum of Alzheimer’s disease neuropathology: combined data from 13 community-based or population-based autopsy cohorts67
Large-scale pathway specific polygenic risk and transcriptomic community network analysis identifies novel functional pathways in Parkinson disease67
Analyzing microglial phenotypes across neuropathologies: a practical guide65
Exosomes induce endolysosomal permeabilization as a gateway by which exosomal tau seeds escape into the cytosol64
The subcellular arrangement of alpha-synuclein proteoforms in the Parkinson’s disease brain as revealed by multicolor STED microscopy63
The role of hnRNPs in frontotemporal dementia and amyotrophic lateral sclerosis60
Characterizing tau deposition in chronic traumatic encephalopathy (CTE): utility of the McKee CTE staging scheme59
Inhibition of Bruton’s tyrosine kinase interferes with pathogenic B-cell development in inflammatory CNS demyelinating disease56
TERT promoter mutation status is necessary and sufficient to diagnose IDH-wildtype diffuse astrocytic glioma with molecular features of glioblastoma56
Multiple system atrophy-associated oligodendroglial protein p25α stimulates formation of novel α-synuclein strain with enhanced neurodegenerative potential53
Neuron-specific activation of necroptosis signaling in multiple sclerosis cortical grey matter53
Extrinsic immune cell-derived, but not intrinsic oligodendroglial factors contribute to oligodendroglial differentiation block in multiple sclerosis53
Evidence of distinct α-synuclein strains underlying disease heterogeneity53
Primary mismatch repair deficient IDH-mutant astrocytoma (PMMRDIA) is a distinct type with a poor prognosis51
Physiological and pathological functions of TMEM106B: a gene associated with brain aging and multiple brain disorders50
TDP-43 transports ribosomal protein mRNA to regulate axonal local translation in neuronal axons50
Pediatric bithalamic gliomas have a distinct epigenetic signature and frequent EGFR exon 20 insertions resulting in potential sensitivity to targeted kinase inhibition49
White matter microglia heterogeneity in the CNS47
Frontal white matter lesions in Alzheimer’s disease are associated with both small vessel disease and AD-associated cortical pathology47
Enhanced axonal response of mitochondria to demyelination offers neuroprotection: implications for multiple sclerosis47
Perivascular space dilation is associated with vascular amyloid-β accumulation in the overlying cortex47
Atypical teratoid/rhabdoid tumors (ATRTs) with SMARCA4 mutation are molecularly distinct from SMARCB1-deficient cases46
Structure of Tau filaments in Prion protein amyloidoses46
Infratentorial IDH-mutant astrocytoma is a distinct subtype45
Interleukin-1 promotes autoimmune neuroinflammation by suppressing endothelial heme oxygenase-1 at the blood–brain barrier45
Meningeal inflammation in multiple sclerosis induces phenotypic changes in cortical microglia that differentially associate with neurodegeneration45
Patient-derived orthotopic xenografts of pediatric brain tumors: a St. Jude resource45
SFPQ and Tau: critical factors contributing to rapid progression of Alzheimer’s disease45
R1441G but not G2019S mutation enhances LRRK2 mediated Rab10 phosphorylation in human peripheral blood neutrophils44
Biological sex and DNA repair deficiency drive Alzheimer’s disease via systemic metabolic remodeling and brain mitochondrial dysfunction44
The coarse-grained plaque: a divergent Aβ plaque-type in early-onset Alzheimer’s disease44
Clinical and molecular heterogeneity of pineal parenchymal tumors: a consensus study43
ETMR: a tumor entity in its infancy43
Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS43
Lewy pathology of the esophagus correlates with the progression of Lewy body disease: a Japanese cohort study of autopsy cases42
Insulin-like growth factor 2 (IGF2) protects against Huntington’s disease through the extracellular disposal of protein aggregates42
Pyroptosis in Alzheimer’s disease: cell type-specific activation in microglia, astrocytes and neurons40
Selective vulnerability of inhibitory networks in multiple sclerosis39
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 neuroinflammation38
Genome-wide meta-analysis for Alzheimer’s disease cerebrospinal fluid biomarkers37
LATE-NC staging in routine neuropathologic diagnosis: an update37
Profiling the neurovascular unit unveils detrimental effects of osteopontin on the blood–brain barrier in acute ischemic stroke37
Integrated molecular and clinical analysis of low-grade gliomas in children with neurofibromatosis type 1 (NF1)36
PATZ1 fusions define a novel molecularly distinct neuroepithelial tumor entity with a broad histological spectrum36
Neuronal activity modulates alpha-synuclein aggregation and spreading in organotypic brain slice cultures and in vivo36
Supratentorial ependymoma in childhood: more than just RELA or YAP36
Maturation of neuronal AD-tau pathology involves site-specific phosphorylation of cytoplasmic and synaptic tau preceding conformational change and fibril formation35
Subgroup and subtype-specific outcomes in adult medulloblastoma35
Upstream open reading frame with NOTCH2NLC GGC expansion generates polyglycine aggregates and disrupts nucleocytoplasmic transport: implications for polyglycine diseases34
Post-mortem analyses of PiB and flutemetamol in diffuse and cored amyloid-β plaques in Alzheimer’s disease34
Making sense of missense variants in TTN-related congenital myopathies34
Recurrent fusions in PLAGL1 define a distinct subset of pediatric-type supratentorial neuroepithelial tumors34
The existence of Aβ strains and their potential for driving phenotypic heterogeneity in Alzheimer’s disease34
Rainwater Charitable Foundation criteria for the neuropathologic diagnosis of progressive supranuclear palsy34
Cryo-EM structures of prion protein filaments from Gerstmann–Sträussler–Scheinker disease32
A multifactorial model of pathology for age of onset heterogeneity in familial Alzheimer’s disease32
Frequent inactivating mutations of the PBAF complex gene PBRM1 in meningioma with papillary features32
Seizure-mediated iron accumulation and dysregulated iron metabolism after status epilepticus and in temporal lobe epilepsy31
Age-associated insolubility of parkin in human midbrain is linked to redox balance and sequestration of reactive dopamine metabolites31
Non-IDH1-R132H IDH1/2 mutations are associated with increased DNA methylation and improved survival in astrocytomas, compared to IDH1-R132H mutations31
Clear cell meningiomas are defined by a highly distinct DNA methylation profile and mutations in SMARCE131
Cross-platform transcriptional profiling identifies common and distinct molecular pathologies in Lewy body diseases31
Complement-associated loss of CA2 inhibitory synapses in the demyelinated hippocampus impairs memory30
Chronic traumatic encephalopathy (CTE): criteria for neuropathological diagnosis and relationship to repetitive head impacts30
CDKN2A deletion in supratentorial ependymoma with RELA alteration indicates a dismal prognosis: a retrospective analysis of the HIT ependymoma trial cohort30
In vitro amplification of pathogenic tau conserves disease-specific bioactive characteristics30
Distinct tau neuropathology and cellular profiles of an APOE3 Christchurch homozygote protected against autosomal dominant Alzheimer’s dementia30
Altered ceramide metabolism is a feature in the extracellular vesicle-mediated spread of alpha-synuclein in Lewy body disorders30
Lipids, lysosomes and mitochondria: insights into Lewy body formation from rare monogenic disorders30
BTK inhibition limits B-cell–T-cell interaction through modulation of B-cell metabolism: implications for multiple sclerosis therapy29
The proteome of granulovacuolar degeneration and neurofibrillary tangles in Alzheimer’s disease29
Distinct characteristics of limbic-predominant age-related TDP-43 encephalopathy in Lewy body disease28
Prion propagation estimated from brain diffusion MRI is subtype dependent in sporadic Creutzfeldt–Jakob disease28
Multi-omic molecular profiling reveals potentially targetable abnormalities shared across multiple histologies of brain metastasis28
Vesicle trafficking and lipid metabolism in synucleinopathy28
Distinct clinicopathologic clusters of persons with TDP-43 proteinopathy27
Single-cell profiling of myasthenia gravis identifies a pathogenic T cell signature27
Malignant transformation of a polymorphous low grade neuroepithelial tumor of the young (PLNTY)26
Altered oligodendroglia and astroglia in chronic traumatic encephalopathy26
Mosaic trisomy of chromosome 1q in human brain tissue associates with unilateral polymicrogyria, very early-onset focal epilepsy, and severe developmental delay26
Cryo-EM structures of amyloid-β filaments with the Arctic mutation (E22G) from human and mouse brains25
CSF p-tau increase in response to Aβ-type and Danish-type cerebral amyloidosis and in the absence of neurofibrillary tangles25
Accumulation of TMEM106B C-terminal fragments in neurodegenerative disease and aging25
Apolipoprotein E regulates lipid metabolism and α-synuclein pathology in human iPSC-derived cerebral organoids25
HnRNP K mislocalisation is a novel protein pathology of frontotemporal lobar degeneration and ageing and leads to cryptic splicing24
Epigenomic, genomic, and transcriptomic landscape of schwannomatosis24
HIF-1α is involved in blood–brain barrier dysfunction and paracellular migration of bacteria in pneumococcal meningitis24
Ultrastructural and biochemical classification of pathogenic tau, α-synuclein and TDP-4324
Necrotic reshaping of the glioma microenvironment drives disease progression23
Trafficking of the glutamate transporter is impaired in LRRK2-related Parkinson’s disease23
Heterogeneity in α-synuclein fibril activity correlates to disease phenotypes in Lewy body dementia23
Glioblastomas with primitive neuronal component harbor a distinct methylation and copy-number profile with inactivation of TP53, PTEN, and RB123
APOE4 exacerbates α-synuclein seeding activity and contributes to neurotoxicity in Alzheimer’s disease with Lewy body pathology23
Phenotypic diversity of genetic Creutzfeldt–Jakob disease: a histo-molecular-based classification23
Reduction of advanced tau-mediated memory deficits by the MAP kinase p38γ23
Tau immunotherapy is associated with glial responses in FTLD-tau23
Impairment of the mitochondrial one-carbon metabolism enzyme SHMT2 causes a novel brain and heart developmental syndrome23
MEF2 impairment underlies skeletal muscle atrophy in polyglutamine disease23
miR155 regulation of behavior, neuropathology, and cortical transcriptomics in Alzheimer's disease22
Wolframin is a novel regulator of tau pathology and neurodegeneration22
CRISPR deletion of the C9ORF72 promoter in ALS/FTD patient motor neurons abolishes production of dipeptide repeat proteins and rescues neurodegeneration22
Oligodendroglia heterogeneity in the human central nervous system22
The oncogenic fusion landscape in pediatric CNS neoplasms22
Retinal pathological features and proteome signatures of Alzheimer’s disease21
MOG-expressing teratoma followed by MOG-IgG-positive optic neuritis21
Targeting cancer stem cells in medulloblastoma by inhibiting AMBRA1 dual function in autophagy and STAT3 signalling21
Developmental malformations in Huntington disease: neuropathologic evidence of focal neuronal migration defects in a subset of adult brains21