Neuropathology and Applied Neurobiology

Article	Citations
Intracerebral endotheliitis and microbleeds are neuropathological features of COVID‐19	95
Dysfunction of the blood–brain barrier in Alzheimer's disease: Evidence from human studies	75
Classification of diseases with accumulation of Tau protein	47
Deep learning‐based model for diagnosing Alzheimer's disease and tauopathies	44
Systematic review of human post‐mortem immunohistochemical studies and bioinformatics analyses unveil the complexity of astrocyte reaction in Alzheimer's disease	43
Cell senescence in neuropathology: A focus on neurodegeneration and tumours	42
Loss of the metabolism and sleep regulating neuronal populations expressing orexin and oxytocin in the hypothalamus in amyotrophic lateral sclerosis	36
Distinct brain‐derived TDP‐43 strains from FTLD‐TDP subtypes induce diverse morphological TDP‐43 aggregates and spreading patterns in vitro and in vivo	32
Impaired brain insulin signalling in Parkinson's disease	30
A case series of Diffuse Glioneuronal Tumours with Oligodendroglioma‐like features and Nuclear Clusters (DGONC)	29
Identification of intraneuronal amyloid beta oligomers in locus coeruleus neurons of Alzheimer’s patients and their potential impact on inhibitory neurotransmitter receptors and neuronal excitability	27
Utrophin modulator drugs as potential therapies for Duchenne and Becker muscular dystrophies	26
A systems‐level analysis highlights microglial activation as a modifying factor in common epilepsies	26
Machine learning‐based decision tree classifier for the diagnosis of progressive supranuclear palsy and corticobasal degeneration	23
Robust methylation‐based classification of brain tumours using nanopore sequencing	22
Correlations in post‐mortem imaging‐histopathology studies of sporadic human cerebral small vessel disease: A systematic review	21
NOTCH3 variant position is associated with NOTCH3 aggregation load in CADASIL vasculature	21
The S1PR2‐CCL2‐BDNF‐TrkB pathway mediates neuroinflammation and motor incoordination in hyperammonaemia	20
Neurodegenerative movement disorders: An epigenetics perspective and promise for the future	20
TRAPPC11‐related muscular dystrophy with hypoglycosylation of alpha‐dystroglycan in skeletal muscle and brain	20
Balloon cells promote immune system activation in focal cortical dysplasia type 2b	19
MiR‐142‐3p regulates synaptopathy‐driven disease progression in multiple sclerosis	19
Expression pattern of perilipins in human brain during aging and in Alzheimer's disease	19
Neutrophil extracellular traps (NETs) infiltrate haematoma and surrounding brain tissue after intracerebral haemorrhage: A post‐mortem study	18
Impaired myelin production due to an intrinsic failure of oligodendrocytes in mTORpathies	18

Olfactory bulb SARS‐CoV‐2 infection is not paralleled by the presence of virus in other central nervous system areas	18
Non‐coding regulatory elements: Potential roles in disease and the case of epilepsy	18
DNA methylation profiling improves routine diagnosis of paediatric central nervous system tumours: A prospective population‐based study	17
Molecular pathophysiology of human MICU1 deficiency	17
Intranuclear inclusions in skin biopsies are not limited to neuronal intranuclear inclusion disease but can also be seen in oculopharyngodistal myopathy	17
The autophagy‐enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado–Joseph disease	17
Arteriolar neuropathology in cerebral microvascular disease	17
Concurrent tau pathologies in frontotemporal lobar degeneration with TDP‐43 pathology	16
Sex differences in the neuropathological hallmarks of Alzheimer’s disease: focus on cognitively intact elderly individuals	16
CD47 promotes the proliferation and migration of adamantinomatous craniopharyngioma cells by activating the MAPK/ERK pathway, and CD47 blockade facilitates microglia‐mediated phagocytosis	16
HnRNP K mislocalisation in neurons of the dentate nucleus is a novel neuropathological feature of neurodegenerative disease and ageing	16
RAPGEF2 mediates oligomeric Aβ‐induced synaptic loss and cognitive dysfunction in the 3xTg‐AD mouse model of Alzheimer’s disease	16
Proteomic characterisation of polyglucosan bodies in skeletal muscle in RBCK1 deficiency	15
Faster disease progression in Parkinson’s disease with type 2 diabetes is not associated with increased α‐synuclein, tau, amyloid‐β or vascular pathology	15
Tumour‐associated CD204+ microglia/macrophages accumulate in perivascular and perinecrotic niches and correlate with an interleukin‐6‐enriched inflammatory profile in glioblastoma	15
BRAIN UK: Accessing NHS tissue archives for neuroscience research	15
Neuron types in the primate striatum: Stereological analysis of projection neurons and interneurons in control and parkinsonian monkeys	14
Potent T cell‐mediated anti‐inflammatory role of the selective CB2 agonist lenabasum in multiple sclerosis	14
Irisin treatment lowers levels of phosphorylated tau in the hippocampus of pre‐symptomatic female but not male htau mice	14
A rare case of paediatric astroblastoma with concomitant MN1‐GTSE1 and EWSR1‐PATZ1 gene fusions altering management	14
G protein‐coupled receptor kinases are associated with Alzheimer's disease pathology	14
The prognostic significance of clinicopathological features in meningiomas: Microscopic brain invasion can predict patient outcome in otherwise benign meningiomas	13
The blood–CSF–brain route of neurological disease: The indirect pathway into the brain	13
Lipid alterations in human frontal cortex in ALS‐FTLD‐TDP43 proteinopathy spectrum are partly related to peroxisome impairment	13
Characterisation of early ultrastructural changes in the cerebral white matter of CADASIL small vessel disease using high‐pressure freezing/freeze‐substitution	12
Old age genetically confirmed frontotemporal lobar degeneration with TDP‐43 has limbic predominant TDP‐43 deposition	12
Emerging roles for the YAP/TAZ transcriptional regulators in brain tumour pathology and targeting options	12
L‐DOPA regulates α‐synuclein accumulation in experimental parkinsonism	12
Anaplastic ganglioglioma—A diagnosis comprising several distinct tumour types	12
Accurate calling of KIAA1549‐BRAF fusions from DNA of human brain tumours using methylation array‐based copy number and gene panel sequencing data	12
Protein homeostasis in LGMDR9 (LGMD2I) – The role of ubiquitin–proteasome and autophagy–lysosomal system	12
Pathogenic variants of Valosin‐containing protein induce lysosomal damage and transcriptional activation of autophagy regulators in neuronal cells	11
Heterozygous APOE Christchurch in familial Alzheimer’s disease without mutations in other Mendelian genes	11
AKT1E17K‐mutated meningioma cell lines respond to treatment with the AKT inhibitor AZD5363	11
Telomeric alterations in the default mode network during the progression of Alzheimer’s disease: Selective vulnerability of the precuneus	11
Diagnostic accuracy of 1p/19q codeletion tests in oligodendroglioma: A comprehensive meta‐analysis based on a Cochrane systematic review	11
Transcriptional signatures of synaptic vesicle genes define myotonic dystrophy type I neurodegeneration	11
The severity of behavioural symptoms in FTD is linked to the loss of GABRQ‐expressing VENs and pyramidal neurons	11
MOBP and HIP1 in multiple system atrophy: New α‐synuclein partners in glial cytoplasmic inclusions implicated in the disease pathogenesis	11
Seizure activity and brain damage in a model of focal non‐convulsive status epilepticus	11
Capillary pathology with prominent basement membrane reduplication is the hallmark histopathological feature of scleromyositis	11
ALK‐rearranged histiocytosis: Report of two cases with involvement of the central nervous system	10
Pathological substrate of memory impairment in multiple system atrophy	10
The contribution of brain banks to knowledge discovery in amyotrophic lateral sclerosis: A systematic review	10
Splicing factor proline and glutamine rich intron retention, reduced expression and aggregate formation are pathological features of amyotrophic lateral sclerosis	10
Cortical morphometric vulnerability to generalised epilepsy reflects chromosome‐ and cell type‐specific transcriptomic signatures	10
Automated immunofluorescence analysis for sensitive and precise dystrophin quantification in muscle biopsies	10
Alpha adaptins show isoform‐specific association with neurofibrillary tangles in Alzheimer's disease	10
Combinatorial model of amyloid β and tau reveals synergy between amyloid deposits and tangle formation	9
Neuropathology of genetically defined malformations of cortical development—A systematic literature review	9

Cutaneous sensory and autonomic denervation in progressive supranuclear palsy	9
Respiratory chain dysfunction in perifascicular muscle fibres in patients with dermatomyositis is associated with mitochondrial DNA depletion	9
Delineating selective vulnerability of inhibitory interneurons in Alpers' syndrome	9
The desmin mutation R349P increases contractility and fragility of stem cell‐generated muscle micro‐tissues	9
Prodromal sensory neuropathy in Pink1−/−SNCAA53T double mutant Parkinson mice	9
Cauda equina paragangliomas express HOXB13	9
Genetic knockdown of Klk8 has sex‐specific multi‐targeted therapeutic effects on Alzheimer’s pathology in mice	9
Post‐treatment hypermutation in a recurrent diffuse glioma with H3.3 p.G34 Mutation	9
Caspase‐6‐cleaved tau is relevant in Alzheimer's disease and marginal in four‐repeat tauopathies: Diagnostic and therapeutic implications	8
Treatment with the copper compound CuATSM has no significant effect on motor neuronal pathology in patients with ALS	8
CircRNA SRRM4 affects glucose metabolism by regulating PKM alternative splicing via SRSF3 deubiquitination in epilepsy	8
Identifying cellular signalling molecules in developmental disorders of the brain: Evidence from focal cortical dysplasia and tuberous sclerosis	8
Expression, prognostic significance and therapeutic implications of PD‐L1 in gliomas	8
Implementation of TERT promoter mutations improve prognostication of the WHO classification in meningioma	8
Epigenetic age acceleration is associated with oligodendrocyte proportions in MSA and control brain tissue	8
Early loss of locus coeruleus innervation promotes cognitive and neuropathological changes before amyloid plaque deposition in a transgenic rat model of Alzheimer's disease	8
Advanced molecular pathology for rare tumours: A national feasibility study and model for centralised medulloblastoma diagnostics	8
Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model	8
Low‐grade epilepsy‐associated neuroepithelial tumours with a prominent oligodendroglioma‐like component: The diagnostic challenges	8
Rosette‐forming glioneuronal tumours are midline, FGFR1‐mutated tumours	8
Brain TDP‐43 pathology in corticobasal degeneration: Topographical correlation with neuronal loss	8
Gonadotroph tumours with a low SF‐1 labelling index are more likely to recur and are associated with enrichment of the PI3K‐AKT pathway	8
Axonal response of mitochondria to demyelination and complex IV activity within demyelinated axons in experimental models of multiple sclerosis	8
Activating ATF6 in spinal muscular atrophy promotes SMN expression and motor neuron survival through the IRE1α‐XBP1 pathway	7
Optimised tissue clearing minimises distortion and destruction during tissue delipidation	7
Extracellular tau oligomers affect extracellular glutamate handling by astrocytes through downregulation of GLT‐1 expression and impairment of NKA1A2 function	7
Dysregulation of miR‐543 in Parkinson's disease: Impact on the neuroprotective gene SIRT1	7
Recurrent posterior fossa group A (PFA) ependymoma in a young child with constitutional mismatch repair deficiency (CMMRD)	7
Paediatric astroblastoma‐like neuroepithelial tumour of the spinal cord with a MAMLD1‐BEND2 rearrangement	7
TGF‐β activates pericytes via induction of the epithelial‐to‐mesenchymal transition protein SLUG in glioblastoma	7
Cytoplasmic HDAC4 recovers synaptic function in the 3×Tg mouse model of Alzheimer's disease	7
A TOR1AIP1 variant segregating with an early onset limb girdle myasthenia—Support for the role of LAP1 in NMJ function and disease	7
Blinded review of hippocampal neuropathology in sudden unexplained death in childhood reveals inconsistent observations and similarities to explained paediatric deaths	7
Morphological differences between dementia with Lewy bodies and Parkinson's disease‐dementia	7
Primary age‐related tauopathy in a Finnish population‐based study of the oldest old (Vantaa 85+)	7
WNT‐activated, MYC‐amplified medulloblastoma displaying intratumoural heterogeneity	6
m6A mRNA methylation in human brain is disrupted in Lewy body disorders	6
GAB1 overexpression identifies hedgehog‐activated anterior skull base meningiomas	6
An autopsy case of pure nigropathy with TUBA4A nonsense mutation	6
Novel naturally occurring autoantibodies attenuate α‐synuclein pathology in a mouse model of Parkinson's disease	6
Early changes in visuospatial episodic memory can help distinguish primary age‐related tauopathy from Alzheimer’s disease	6
Spatial molecular profiling of a central nervous system low‐grade diffusely infiltrative tumour with INI1 deficiency featuring a high‐grade atypical teratoid/rhabdoid tumour component	6
The interaction of insoluble Amyloid‐β with soluble Amyloid‐β dimers decreases Amyloid‐β plaque numbers	6
Past antihypertensive medication use is associated with lower levels of small vessel disease and lower Aβ plaque stage in the brains of older individuals	6
A high‐throughput single‐cell RNA expression profiling method identifies human pericyte markers	6
CuII(atsm) significantly decreases microglial reactivity in patients with sporadic amyotrophic lateral sclerosis	6
Met166‐Glu168 residues in human PrP β2‐α2 loop account for evolutionary resistance to prion infection	6
Inflammation, fibrosis and skeletal muscle regeneration in LGMDR9 are orchestrated by macrophages	6
Unveiling the identities of null cell tumours: Epigenomics corroborate subtle histological cues in pituitary neuroendocrine tumour/adenoma classification	6
Nanoscale reorganisation of synaptic proteins in Alzheimer's disease	5
Myeloid cell subpopulations compensate each other for Ccr2‐deficiency in glioblastoma	5
Low‐grade diffusely infiltrative tumour (LGDIT), SMARCB1‐mutant: A clinical and histopathological distinct entity showing epigenetic similarity with ATRT‐MYC	5
Identification of the calpain‐generated toxic fragment of ataxin‐3 protein provides new avenues for therapy of Machado–Joseph disease| Spinocerebellar ataxia type 3	5
MicroRNA‐34a activation in tuberous sclerosis complex during early brain development may lead to impaired corticogenesis	5
Identification of a novel heterozygous DYSF variant in a large family with a dominantly‐inherited dysferlinopathy	5
The molecular characteristics of low‐grade and high‐grade areas in desmoplastic infantile astrocytoma/ganglioglioma	5
Lack of difference between amyloid‐beta burden at gyral crests and sulcal depths in diverse neurodegenerative diseases	5
Novel autophagic vacuolar myopathies: Phenotype and genotype features	5
IDH clonal heterogeneity segregates a subgroup of non‐1p/19q codeleted gliomas with unfavourable clinical outcome	5
Intrathecal amyloid‐beta oligomer administration increases tau phosphorylation in the medial temporal lobe in the African green monkey: A nonhuman primate model of Alzheimer's disease	5
Abundant copathologies of polyglucosan bodies, frontotemporal lobar degeneration with TDP‐43 inclusions and ageing‐related tau astrogliopathy in a family with aGBE1mutation	5
The clinico‐pathological characterisation of focal cortical dysplasia type IIb genetically defined by MTOR mosaicism	5
Comprehensive analysis of intratumoural heterogeneity of somatic copy number alterations in diffuse glioma reveals clonality‐dependent prognostic patterns	4
Atypical astroglial pTDP‐43 pathology in astroglial predominant tauopathy	4
A novel TRMT5 mutation causes a complex inherited neuropathy syndrome: The role of nerve pathology in defining a demyelinating neuropathy	4
The SPTLC1 p.S331 mutation bridges sensory neuropathy and motor neuron disease and has implications for treatment	4
Absence of tissue transglutaminase reduces amyloid‐beta pathology in APP23 mice	4
Long‐standing multiple system atrophy‐Parkinsonism with limbic and FTLD‐type α‐synuclein pathology	4
Interfering with lysophosphatidic acid receptor edg2/lpa1 signalling slows down disease progression in SOD1‐G93A transgenic mice	4
The genomic landscape of dysembryoplastic neuroepithelial tumours and a comprehensive analysis of recurrent cases	4
RNA sequencing of peripheral blood in amyotrophic lateral sclerosis reveals distinct molecular subtypes: Considerations for biomarker discovery	4
Linking epigenetic signature and metabolic phenotype in IDH mutant and IDH wildtype diffuse glioma	4
What to do with unusual TDP‐43 proteinopathy cases?	4
Muscle biopsy practices in the evaluation of neuromuscular disease: A systematic literature review	4
N471D WASH complex subunit strumpellin knock‐in mice display mild motor and cardiac abnormalities and BPTF and KLHL11 dysregulation in brain tissue	4
Distinguishing post‐translational modifications in dominantly inherited frontotemporal dementias: FTLD‐TDP Type A (GRN) vs Type B (C9orf72)	4
CD8 T‐cell‐mediated cerebellitis directed against Purkinje cell antigen after ipilimumab for small cell lung cancer	4
MBNL‐dependent impaired development within the neuromuscular system in myotonic dystrophy type 1	4
Inflammatory bowel disease induces pathological α‐synuclein aggregation in the human gut and brain	4
Motor neuron involvement threatens survival in spinocerebellar ataxia type 1	4

Evaluating the efficacy of few‐shot learning for GPT‐4Vision in neurodegenerative disease histopathology: A comparative analysis with convolutional neural network model	4
Neuronal infection is a major pathogenetic mechanism and cause of fatalities in human acute Nipah virus encephalitis	4
TDP‐43 proteinopathy occurs independently of autophagic substrate accumulation and underlies nuclear defects in Niemann‐Pick C disease	4
Schwann cells and myelin in human peripheral nerve: Major protein components vary with age, axon size and pathology	3
Muscleblind‐like 2 knockout shifts adducin 1 isoform expression and alters dendritic spine dynamics of cortical neurons during brain development	3
Insights into the pathological basis of dementia from population‐based neuropathology studies	3
Neurotherapeutic implications of sense and respond strategies generated by astrocytes and astrocytic tumours to combat pH mechanical stress	3
Evidence of neural crest cell origin of a DICER1 mutant CNS sarcoma in a child with DICER1 syndrome and NRAS‐mutant neurocutaneous melanosis	3
First report of medulloblastoma in a patient with MUTYH‐associated polyposis	3
Myostatin in idiopathic inflammatory myopathies: Serum assessment and disease activity	3
Time to focus on circulating nucleic acids for diagnosis and monitoring of gliomas: A systematic review of their role as biomarkers	3
microRNA‐based predictor for diagnosis of frontotemporal dementia	3
Image‐based deep learning reveals the responses of human motor neurons to stress and VCP‐related ALS	3
A threshold for mitotic activity and post‐surgical residual volume defines distinct prognostic groups for astrocytoma IDH‐mutant	3
Genetic inhibition of PDK1 robustly reduces plaque deposition and ameliorates gliosis in the 5×FAD mouse model of Alzheimer's disease	3
Intelligence quotient–genotype association in dystrophinopathies: A systematic review and meta‐analysis	3
TAPPing into the potential of inducible tau/APP transgenic mice	3
Molecular landscape of IDH‐wild‐type, H3‐wild‐type glioblastomas of adolescents and young adults	3
Hippocampal subfield pathologic Burden in Lewy body diseases versus Alzheimer’s disease	3
Effects of mutant huntingtin in oxytocin neurons on non‐motor features of Huntington's disease	2
Paths to hippocampal damage in neuromyelitis optica spectrum disorders	2
Target formation in muscle fibres indicates reinnervation – A proteomic study in muscle samples from peripheral neuropathies	2
A centronuclear myopathy‐causing mutation in dynamin‐2 disrupts neuronal morphology and excitatory synaptic transmission in a murine model of the disease	2
Oxygen treatment reduces neurological deficits and demyelination in two animal models of multiple sclerosis	2
Morphological alterations of the neuronal Golgi apparatus upon seizures	2
Simultaneous Nbs1 and p53 inactivation in neural progenitors triggers high‐grade gliomas	2
Effects of mutant huntingtin inactivation on Huntington disease‐related behaviours in the BACHD mouse model	2
Neuromuscular junction denervation and terminal Schwann cell loss in the hTDP‐43 overexpression mouse model of amyotrophic lateral sclerosis	2
A gene dosage‐dependent effect unveils NBS1 as both a haploinsufficient tumour suppressor and an essential gene for SHH‐medulloblastoma	2
Regulation of CNS pathology by Serpina3n/SERPINA3: The knowns and the puzzles	2
NLRP3 promotes radiation‐induced brain injury by regulating microglial pyroptosis	2
Tuberous sclerosis complex‐1 (TSC1) contributes to selective neuronal vulnerability in Alzheimer's disease	2
Alzheimer's disease pathology concomitant with memory impairment in late‐onset multiple system atrophy	2
Identifying diagnostic and prognostic factors in cerebral amyloid angiopathy‐related inflammation: A systematic analysis of published and seven new cases	2
Successful plasmapheresis and immunoglobulin treatment for severe lipid storage myopathy: Doing the right thing for the wrong reason	2
Immune system involvement in neuronal intranuclear inclusion disease	2
Molecular characterisation of sporadic endolymphatic sac tumours and comparison to von Hippel–Lindau disease‐related tumours	2
Different patterns of hippocampal subfield pathology in Lewy body disease and Alzheimer's disease	2
Progressive hemispheric atrophy in HIV: A Rasmussen's‐like variant of CD8 encephalitis?	2