OOIR: Observatory of International Research

Papers

(The median citation count of Neuropathology and Applied Neurobiology is 1. 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 2021-11-01 to 2025-11-01.)

Article	Citations
Intelligence quotient–genotype association in dystrophinopathies: A systematic review and meta‐analysis	133
Role of Astrocytic and Microglial Phenotype in the Biology of Hippocampal Sclerosis	62
Neuropathological Characterisation of McLeod Syndrome With a Proposed New Grading System	55
Novel naturally occurring autoantibodies attenuate α‐synuclein pathology in a mouse model of Parkinson's disease	33
Paediatric astroblastoma‐like neuroepithelial tumour of the spinal cord with a MAMLD1‐BEND2 rearrangement	32
Oral Abstracts	28
Editorial	26
An autopsy case of late‐onset spinocerebellar atrophy type 14	24
Neuronal infection is a major pathogenetic mechanism and cause of fatalities in human acute Nipah virus encephalitis	24
	24
	23
Author Index	22
Neuronal and astrocytic tetraploidy is increased in drug‐resistant epilepsy	21
Anaplastic ganglioglioma—A diagnosis comprising several distinct tumour types	21
A novel SREBF1::NACC1 gene fusion in an unclassifiable intracranial tumour	21
The S1PR2‐CCL2‐BDNF‐TrkB pathway mediates neuroinflammation and motor incoordination in hyperammonaemia	20
Rapid bacterial identification from formalin‐fixed paraffin‐embedded neuropathology specimens using 16S rDNA nanopore sequencing	18
Erratum	18
Response letter: Complexities in pericyte markers	17
Myostatin in idiopathic inflammatory myopathies: Serum assessment and disease activity	17
Myopathology and Immune Profile of Granulomatous Myositis in Sarcoid Myopathy	17
Synaptic gene expression changes in frontotemporal dementia due to the MAPT 10 + 16 mutation	16
A standardised protocol for blood and cerebrospinal fluid collection and processing for biomarker research in ataxia	16
Analysing cerebrospinal fluid with explainable deep learning: From diagnostics to insights	16
124th Meeting of the British Neuropathological Society The View, Royal College of Surgeons of England, Lincoln's Inn Fields, London	16

Genetic inhibition of PDK1 robustly reduces plaque deposition and ameliorates gliosis in the 5×FAD mouse model of Alzheimer's disease	16
Issue Information	15
Concurrent tau pathologies in frontotemporal lobar degeneration with TDP‐43 pathology	15
Perineuronal nets are phagocytosed by MMP‐9 expressing microglia and astrocytes in the SOD1^G93A ALS mouse model	15
	15
Non‐coding regulatory elements: Potential roles in disease and the case of epilepsy	15
Cover Image, Volume 50, Issue 3	15
	14
Fibrodysplasia ossificans progressiva: Histopathological implications of aberrant bone morphogenic protein signalling for CNS dysgenesis	14
Application of Oral Mucosal Epithelial Cells in Noninvasive Pathological Diagnosis of Neuronal Intranuclear Inclusion Disease	14
Evidence of neural crest cell origin of a DICER1 mutant CNS sarcoma in a child with DICER1 syndrome and NRAS‐mutant neurocutaneous melanosis	14
Adult‐onset Alexander disease with unusual inflammatory features and a novel GFAP mutation in two patients	14
Low‐grade epilepsy‐associated neuroepithelial tumours with a prominent oligodendroglioma‐like component: The diagnostic challenges	14
Epigenetic age acceleration is associated with oligodendrocyte proportions in MSA and control brain tissue	14
Phenotypic and epigenetic heterogeneity in FGFR2‐fused glial and glioneuronal tumours	14
m⁶A mRNA methylation in human brain is disrupted in Lewy body disorders	14
Microglia induce an interferon‐stimulated gene expression profile in glioblastoma and increase glioblastoma resistance to temozolomide	13
	13
RNA sequencing of peripheral blood in amyotrophic lateral sclerosis reveals distinct molecular subtypes: Considerations for biomarker discovery	13
TDP‐43 pathology and functional deficits in wild‐type and ALS/FTD mutant cyclin F mouse models	13
Robust methylation‐based classification of brain tumours using nanopore sequencing	13
The blood–CSF–brain route of neurological disease: The indirect pathway into the brain	12
Hippocampal dentate granule cells in temporal lobe epilepsy: A morphometry and transcriptomic study	12
Comprehensive analysis of intratumoural heterogeneity of somatic copy number alterations in diffuse glioma reveals clonality‐dependent prognostic patterns	12
Intramuscular Nerve Bundles Reflect TDP‐43 Pathology in the Medulla and Spinal Cord of ALS Patients	12
Is islet amyloid polypeptide indeed expressed in the human brain?	12
126^th Meeting of the British Neuropathological Society The View, Royal College of Surgeons of England, Lincoln's Inn Fields, London	12
The genomic landscape of dysembryoplastic neuroepithelial tumours and a comprehensive analysis of recurrent cases	12
Issue Information	12
WNT‐activated, MYC‐amplified medulloblastoma displaying intratumoural heterogeneity	12
Neurotherapeutic implications of sense and respond strategies generated by astrocytes and astrocytic tumours to combat pH mechanical stress	12
	12
Axonal response of mitochondria to demyelination and complex IV activity within demyelinated axons in experimental models of multiple sclerosis	11
Interferon‐gamma contributes to disease progression in the Ndufs4(−/−) model of Leigh syndrome	11
Fibroblast growth factor 9 (FGF9)‐mediated neurodegeneration: Implications for progressive multiple sclerosis?	11
	10
Nanopore sequencing identifies Borrelia miyamotoi as an unexpected cause of meningitis after B cell depletion	10
Pathological Characterisation of Posterior Cortical Atrophy in Comparison With Amnestic Alzheimer's Disease	10
AKT1^E17K‐mutated meningioma cell lines respond to treatment with the AKT inhibitor AZD5363	9
Atypical teratoid/rhabdoid tumour‐TYR subtype arising in the setting of germline ring chromosome 22: An uncommon form of tumour predisposition	9
	9
Issue Information	9
Muscle biopsy practices in the evaluation of neuromuscular disease: A systematic literature review	9
HnRNP K mislocalisation in neurons of the dentate nucleus is a novel neuropathological feature of neurodegenerative disease and ageing	9
Corrigendum	9
Issue Information	9
Amino‐terminally elongated Aβ peptides are generated by the secreted metalloprotease ADAMTS4 and deposit in a subset of Alzheimer's disease brains	9
Regional redistribution of CB1 cannabinoid receptors in human foetal brains with Down's syndrome and their functional modifications in Ts65Dn^+/+ mice	8
The SPTLC1 p.S331 mutation bridges sensory neuropathy and motor neuron disease and has implications for treatment	8
Letter to the editor	8

Quantitative cellular changes in multiple system atrophy brains	8
Proteomic profiling of polyglucosan bodies associated with glycogenin‐1 deficiency in skeletal muscle	8
Diagnostic accuracy of 1p/19q codeletion tests in oligodendroglioma: A comprehensive meta‐analysis based on a Cochrane systematic review	8
Low‐grade diffusely infiltrative tumour (LGDIT), SMARCB1‐mutant: A clinical and histopathological distinct entity showing epigenetic similarity with ATRT‐MYC	8
Corrigendum	8
Unfolded protein response markers Grp78 and eIF2alpha are upregulated with increasing alpha‐synuclein levels in Lewy body disease	8
	7
Issue Information	7
Regulation of CNS pathology by Serpina3n/SERPINA3: The knowns and the puzzles	7
Ageing‐related tau astrogliopathy severely affecting the substantia nigra	7
Cytoplasmic HDAC4 recovers synaptic function in the 3×Tg mouse model of Alzheimer's disease	7
Issue Information	7
GFAP expression in the brain during human postnatal development	7
Adult brain tumour research in 2024: Status, challenges and recommendations	7
LMNB1‐duplication mediated nuclear architecture alteration and demyelination of cerebral white matter in a patient with ADLD	6
Cerebellar phenotypes in germline PTEN mutation carriers	6
Soluble amyloid‐β dimers are resistant to amyloid‐β prion conversion in vivo suggesting antiprion properties	6
Dysfunction of the blood–brain barrier in Alzheimer's disease: Evidence from human studies	6
Mechanisms of COVID‐19‐associated olfactory dysfunction	6
A novel homozygous nonsense variant in COL12A1 causes myopathic Ehlers‐Danlos syndrome: A case report and literature review	6
Multiplex Immunofluorescent Analysis of Alpha‐Synuclein in Nigral Lewy Bodies With Heat‐Induced Antibody Stripping Reveals an Intricate Multilayered Structure	6
Identification of a novel heterozygous DYSF variant in a large family with a dominantly‐inherited dysferlinopathy	6
Computational Analysis of SOD1‐G93A Mouse Muscle Biomarkers for Comprehensive Assessment of ALS Progression	5
Cover Image, Volume 50, Issue 1	5
Issue Information	5
Methyl donor supplementation reduces phospho‐Tau, Fyn and demethylated protein phosphatase 2A levels and mitigates learning and motor deficits in a mouse model of tauopathy	5
Refining Muscle Morphometry Through Machine Learning and Spatial Analysis	5
TAF15 and Transportin 1 in Intranuclear Inclusions of Neuronal Intranuclear Inclusion Disease	5
PLAG‐Family Amplified CNS Embryonal Tumour With PLAG1 Immunohistochemical Expression: Expanding the Spectrum of Diagnostic Tools	5
125th Meeting of the British Neuropathological Society The View, Royal College of Surgeons of England, Lincoln's Inn Fields, London	5
Relationships Between Alcohol Intake and Mitochondrial DNA Methylation in the Human Prefrontal Cortex and Nucleus Accumbens	5
Issue Information	5
Cortical morphometric vulnerability to generalised epilepsy reflects chromosome‐ and cell type‐specific transcriptomic signatures	4
The molecular mechanisms that underlie IGHMBP2‐related diseases	4
Ectopic expression of neuronal adenosine kinase, a biomarker in mesial temporal lobe epilepsy without hippocampal sclerosis	4
Standardised TruAI Automated Quantification of Intracellular Neuromelanin Granules in Human Brain Tissue Sections	4
Phosphorylation of MAP 1A regulates hyperphosphorylation of Tau in Alzheimer's disease model	4
Classification of diseases with accumulation of Tau protein	4
	4
Muscleblind‐like 2 knockout shifts adducin 1 isoform expression and alters dendritic spine dynamics of cortical neurons during brain development	4
Metastatic Bifocal Germinoma With Dramatic Early Steroid Response, Utility of Circulating miR‐371a‐3p and Vinblastine Monotherapy Prior to Definitive Craniospinal Irradiation	4
Psychotic symptoms in frontotemporal dementia with TDP‐43 tend to be associated with type B pathology	4
Loss of IDH mutation or secondary tumour manifestation? Evolution of an IDH‐mutant and 1p/19q‐codeleted oligodendroglioma after 15 years of continuous temozolomide treatment and radiotherapy: A case r	4
Distinguishing post‐translational modifications in dominantly inherited frontotemporal dementias: FTLD‐TDP Type A (GRN) vs Type B (C9orf72)	4
Long‐standing multiple system atrophy‐Parkinsonism with limbic and FTLD‐type α‐synuclein pathology	4
Issue Information	4
Artificial intelligence in histopathological image analysis of central nervous system tumours: A systematic review	4
	4
Analysis of a pituitary tumour with histological features of central neurocytoma points towards the emergence of a new entity recognisable by a specific epigenetic signature	4
Simultaneous Nbs1 and p53 inactivation in neural progenitors triggers high‐grade gliomas	4
	3
Oxygen treatment reduces neurological deficits and demyelination in two animal models of multiple sclerosis	3
Skeletal muscle involvement in systemic amyloidosis is often overlooked	3
Unveiling the identities of null cell tumours: Epigenomics corroborate subtle histological cues in pituitary neuroendocrine tumour/adenoma classification	3
Issue Information	3
Testing Meningiomas With Methylation Arrays: Insights and Recommendations From a Large Single‐Centre Study	3
Nanoscale reorganisation of synaptic proteins in Alzheimer's disease	3
Cover Image, Volume 48, Issue 5	3
Neuroinvasion via Peripheral Nerves in Epidemic Viral Encephalitis Caused by Enterovirus, Orthoflavivirus and SARS‐Coronavirus	3
Early loss of locus coeruleus innervation promotes cognitive and neuropathological changes before amyloid plaque deposition in a transgenic rat model of Alzheimer's disease	3
	3
Change in the molecular properties of CH1641 prions after transmission to wild‐type mice: Evidence for a single strain	3
	3
Combinatorial model of amyloid β and tau reveals synergy between amyloid deposits and tangle formation	3
Respiratory chain dysfunction in perifascicular muscle fibres in patients with dermatomyositis is associated with mitochondrial DNA depletion	3
	3
An Intracerebrally‐Infected Mouse Model of Enterovirus A71 Demonstrates Restricted Inter‐Neuronal Spread Within the Brain Parenchyma Despite Strong SCARB2 Expression	3
Pathological substrate of memory impairment in multiple system atrophy	3
	3
Paths to hippocampal damage in neuromyelitis optica spectrum disorders	3
Cu^II(atsm) significantly decreases microglial reactivity in patients with sporadic amyotrophic lateral sclerosis	3
Chronic traumatic encephalopathy neuropathologic change in former Australian rugby players	2
Protein Coaggregation in Caribbean Atypical Parkinsonism: The Contribution of Annonacin	2
Genotype–Phenotype Correlation in Progressive External Ophthalmoplegia: Insights From a Retrospective Analysis	2
Tuberous sclerosis complex‐1 (TSC1) contributes to selective neuronal vulnerability in Alzheimer's disease	2
Post‐mortem 7T MR imaging and neuropathology in middle stage juvenile‐onset Huntington disease: A case report	2
Recurrent posterior fossa group A (PFA) ependymoma in a young child with constitutional mismatch repair deficiency (CMMRD)	2
A Variant of OTUD3 in Early‐Onset Parkinsonism	2
Diffuse infiltrating tumour with the molecular profile of an atypical teratoid rhabdoid tumour (AT/RT SHH‐1B) in an adult patient	2
Pathogenic variants of Valosin‐containing protein induce lysosomal damage and transcriptional activation of autophagy regulators in neuronal cells	2

Investigating key factors underlying neurodegeneration linked to alpha‐synuclein spread	2
Slow disease progression and characteristic TDP‐43 inclusions in a patient with familial amyotrophic lateral sclerosis carrying a TARDBP G357S variant	2
	2
Identical Seeding Characteristics and Cryo‐EM Filament Structures in FTLD‐Synuclein and Typical Multiple System Atrophy	2
Elevated expression of urokinase plasminogen activator in rodent models and patients with cerebral amyloid angiopathy	2
A reassessment of spinal cord pathology in severe infantile spinal muscular atrophy	2
The contribution of brain banks to knowledge discovery in amyotrophic lateral sclerosis: A systematic review	2
A high‐throughput single‐cell RNA expression profiling method identifies human pericyte markers	2
Progressive hemispheric atrophy in HIV: A Rasmussen's‐like variant of CD8 encephalitis?	2
TAPPing into the potential of inducible tau/APP transgenic mice	2
Clinicopathological Characteristics and Immune Microenvironment of Posterior Pituitary Tumours	2
Absence of tissue transglutaminase reduces amyloid‐beta pathology in APP23 mice	1
Identifying diagnostic and prognostic factors in cerebral amyloid angiopathy‐related inflammation: A systematic analysis of published and seven new cases	1
Issue Information	1
Nanoscale reorganisation of synaptic proteins in Alzheimer's disease	1
	1
A case of glioneuronal tumour with ATRX alteration, kinase fusion and anaplastic features showing rapid ependymal and leptomeningeal dissemination	1
Delineating selective vulnerability of inhibitory interneurons in Alpers' syndrome	1
Beyond vacuolar pathology: Multiomic profiling of Danon disease reveals dysfunctional mitochondrial homeostasis	1
Neuron types in the primate striatum: Stereological analysis of projection neurons and interneurons in control and parkinsonian monkeys	1
Abundant copathologies of polyglucosan bodies, frontotemporal lobar degeneration with TDP‐43 inclusions and ageing‐related tau astrogliopathy in a family with aGBE1mutation	1
Roy O. Weller	1
A Homozygous ATP2A2 Variant Alters Sarcoendoplasmic Reticulum Ca²⁺‐ATPase 2 Function in Skeletal Muscle and Causes a Novel Vacuolar Myopathy	1
Collablots: Quantification of Collagen VI Levels and Its Structural Disorganisation in Cell Cultures From Patients With Collagen VI‐Related Dystrophies	1
Interleukin‐6 and interferon‐alpha differentially regulate microglia function	1
Author Index	1
Cover Image, Volume 48, Issue 4	1
The desmin mutation R349P increases contractility and fragility of stem cell‐generated muscle micro‐tissues	1
Issue Information	1
Novel Aspects of Hereditary Spastic Paraplegia: A Clinicopathologic and Biochemical Study of a Patient With a Heterozygous GCH1 Variant	1
Atrophy and Higher Levels of Inflammatory‐Related Markers in the Posterior Cerebellar Lobe Cortex in Chronic Alcohol Use Disorder: A Cross‐Sectional Study	1
DNA methylation‐based classification of glioneuronal tumours synergises with histology and radiology to refine accurate molecular stratification	1
Poster Abstracts	1
TRAPPC11‐related muscular dystrophy with hypoglycosylation of alpha‐dystroglycan in skeletal muscle and brain	1
Brain TDP‐43 pathology in corticobasal degeneration: Topographical correlation with neuronal loss	1
Brain metastasis of a urothelial neuroendocrine carcinoma: A double pitfall for neuropathologists and DNA‐methylation profiling	1
123rd meeting of the British Neuropathological Society	1
Author Index	1
DNA methylation profiling improves routine diagnosis of paediatric central nervous system tumours: A prospective population‐based study	1
Characterisation of the tumour microenvironment in primary and recurrent glioblastomas	1
Three‐Dimensional Visualisation of Blood Vessels in Human Gliomas Using Tissue Clearing and Deep Learning	1
Revisiting the relevance of Hirano bodies in neurodegenerative diseases	1
Myeloid cell subpopulations compensate each other for Ccr2‐deficiency in glioblastoma	1