Humanized tau antibodies promote tau uptake by human microglia without any increase of inflammation
Immunotherapies targeting pathological tau have recently emerged as a promising approach for treatment of neurodegenerative disorders. We have previously showed that the mouse antibody DC8E8 discriminates betw...Acta Neuropathologica Communications 2020 8:74Meningomyeloencephalitis secondary to Mycobacterium haemophilum infection in AIDS
Infections by opportunistic non-tuberculous mycobacteria (NTM) are rising in global incidence. One emerging, slowly growing NTM is Mycobacterium haemophilum, which can cause skin, lung, bone, and soft tissue infe...Acta Neuropathologica Communications 2020 8:73Loss of homeostatic microglial phenotype in CSF1R-related Leukoencephalopathy
Microglia are resident macrophages of the central nervous system, and their unique molecular signature is dependent upon CSF-1 signaling. Previous studies have demonstrated the importance of CSF-1R in survival...Acta Neuropathologica Communications 2020 8:72- Acta Neuropathologica Communications 2020 8:71
Restoration of miR-193a expression is tumor-suppressive in MYC amplified Group 3 medulloblastoma
Medulloblastoma, a highly malignant pediatric brain tumor, consists of four molecular subgroups, namely WNT, SHH, Group 3, and Group 4. The expression of miR-193a, a WNT subgroup-specific microRNA, was found t...Acta Neuropathologica Communications 2020 8:70Correction to: Differential gene methylation and expression of HOX transcription factor family in orbitofacial neurofibroma
An amendment to this paper has been published and can be accessed via the original article.Acta Neuropathologica Communications 2020 8:69The original article was published in Acta Neuropathologica Communications 2020 8:62Transcriptional analyses of adult and pediatric adamantinomatous craniopharyngioma reveals similar expression signatures regarding potential therapeutic targets
Adamantinomatous craniopharyngioma (ACP) is a biologically benign but clinically aggressive lesion that has a significant impact on quality of life. The incidence of the disease has a bimodal distribution, wit...Acta Neuropathologica Communications 2020 8:68Behavioral and electrophysiological evidence for a neuroprotective role of aquaporin-4 in the 5xFAD transgenic mice model
Aquaporin-4 (AQP4) has been suggested to be involved in the pathogenesis of neurodegenerative diseases including Alzheimer’s disease (AD), which may be due to the modulation of neuroinflammation or the impairm...Acta Neuropathologica Communications 2020 8:67Persistent elevation of intrathecal pro-inflammatory cytokines leads to multiple sclerosis-like cortical demyelination and neurodegeneration
Analysis of isolated meninges and cerebrospinal fluid (CSF) of post-mortem MS cases has shown increased gene and protein expression for the pro-inflammatory cytokines: tumour necrosis factor (TNF) and interfer...Acta Neuropathologica Communications 2020 8:66Transmission of ALS pathogenesis by the cerebrospinal fluid
To test the hypothesis that the cerebrospinal fluid (CSF) could provide a spreading route for pathogenesis of amyotrophic lateral sclerosis (ALS), we have examined the effects of intraventricular infusion duri...Acta Neuropathologica Communications 2020 8:65RNA modifications in brain tumorigenesis
RNA modifications are emerging as critical regulators in cancer biology, thanks to their ability to influence gene expression and the predominant protein isoforms expressed during cell proliferation, migration...Acta Neuropathologica Communications 2020 8:64Genetic perspective on the synergistic connection between vesicular transport, lysosomal and mitochondrial pathways associated with Parkinson’s disease pathogenesis
Parkinson’s disease (PD) and atypical parkinsonian syndromes (APS) are symptomatically characterized by parkinsonism, with the latter presenting additionally a distinctive range of atypical features. Although ...Acta Neuropathologica Communications 2020 8:63Differential gene methylation and expression of HOX transcription factor family in orbitofacial neurofibroma
Although most commonly benign, neurofibromas (NFs) can have devastating functional and cosmetic effects in addition to the possibility of malignant transformation. In orbitofacial neurofibromatosis type 1, NFs...Acta Neuropathologica Communications 2020 8:62Distinct molecular patterns of TDP-43 pathology in Alzheimer’s disease: relationship with clinical phenotypes
The co-existence of multiple pathologies and proteins is a common feature in the brains of cognitively impaired elderly individuals. Transactive response DNA-binding protein (TDP-43) has been discovered to acc...Acta Neuropathologica Communications 2020 8:61Association of Aβ with ceramide-enriched astrosomes mediates Aβ neurotoxicity
Amyloid-β (Aβ) associates with extracellular vesicles termed exosomes. It is not clear whether and how exosomes modulate Aβ neurotoxicity in Alzheimer’s disease (AD). We show here that brain tissue and serum f...Acta Neuropathologica Communications 2020 8:60Validation of machine learning models to detect amyloid pathologies across institutions
Semi-quantitative scoring schemes like the Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) are the most commonly used method in Alzheimer’s disease (AD) neuropathology practice. Computationa...Acta Neuropathologica Communications 2020 8:59Enhanced accumulation of N-terminally truncated Aβ with and without pyroglutamate-11 modification in parvalbumin-expressing GABAergic neurons in idiopathic and dup15q11.2-q13 autism
Autism, the most frequent neurodevelopmental disorder of a very complex etiopathology, is associated with dysregulation of cellular homeostatic mechanisms, including processing of amyloid-β precursor protein (...Acta Neuropathologica Communications 2020 8:58Clinical, imaging, and molecular analysis of pediatric pontine tumors lacking characteristic imaging features of DIPG
Diffuse intrinsic pontine glioma (DIPG) is most commonly diagnosed based on imaging criteria, with biopsy often reserved for pontine tumors with imaging features not typical for DIPG (atypical DIPG, ‘aDIPG’). ...Acta Neuropathologica Communications 2020 8:57Unilateral intranigral administration of β-sitosterol β-D-glucoside triggers pathological α-synuclein spreading and bilateral nigrostriatal dopaminergic neurodegeneration in the rat
The spreading and accumulation of α-synuclein and dopaminergic neurodegeneration, two hallmarks of Parkinson’s disease (PD), have been faithfully reproduced in rodent brains by chronic, oral administration of ...Acta Neuropathologica Communications 2020 8:56Common gene expression signatures in Parkinson’s disease are driven by changes in cell composition
The etiology of Parkinson’s disease is largely unknown. Genome-wide transcriptomic studies in bulk brain tissue have identified several molecular signatures associated with the disease. While these studies hav...Acta Neuropathologica Communications 2020 8:55Hippocampal granule cell dispersion: a non-specific finding in pediatric patients with no history of seizures
Chronic epilepsy has been associated with hippocampal abnormalities like neuronal loss, gliosis and granule cell dispersion. The granule cell layer of a normal human hippocampal dentate gyrus is traditionally ...Acta Neuropathologica Communications 2020 8:54A high–throughput digital script for multiplexed immunofluorescent analysis and quantification of sarcolemmal and sarcomeric proteins in muscular dystrophies
The primary molecular endpoint for many Duchenne muscular dystrophy (DMD) clinical trials is the induction, or increase in production, of dystrophin protein in striated muscle. For accurate endpoint analysis, ...Acta Neuropathologica Communications 2020 8:53Simultaneous detection of EGFR amplification and EGFRvIII variant using digital PCR-based method in glioblastoma
Epidermal growth factor receptor (EGFR) amplification and EGFR variant III (EGFRvIII, deletion of exons 2–7) are of clinical interest for glioblastoma. The aim was to develop a digital PCR (dPCR)-based method usi...Acta Neuropathologica Communications 2020 8:52Characterising cellular and molecular features of human peripheral nerve degeneration
Nerve regeneration is a key biological process in those recovering from neural trauma. From animal models it is known that the regenerative capacity of the peripheral nervous system (PNS) relies heavily on the...Acta Neuropathologica Communications 2020 8:51Mitochondrial respiratory chain deficiency correlates with the severity of neuropathology in sporadic Creutzfeldt-Jakob disease
Mitochondrial dysfunction has been implicated in multiple neurodegenerative diseases but remains largely unexplored in Creutzfeldt-Jakob disease. Here, we characterize the mitochondrial respiratory chain at th...Acta Neuropathologica Communications 2020 8:50Induction of aquaporin 4-reactive antibodies in Lewis rats immunized with aquaporin 4 mimotopes
Most cases of neuromyelitis optica spectrum disorders (NMOSD) harbor pathogenic autoantibodies against the water channel aquaporin 4 (AQP4). Binding of these antibodies to AQP4 on astrocytes initiates damage t...Acta Neuropathologica Communications 2020 8:49Foetal onset of EIF2B related disorder in two siblings: cerebellar hypoplasia with absent Bergmann glia and severe hypomyelination
Bi-allelic pathogenic variants in genes of the EIF2B family are responsible for Childhood Ataxia with Central nervous system Hypomyelination/Vanishing White Matter disease, a progressive neurodegenerative diso...Acta Neuropathologica Communications 2020 8:48Abnormal mitosis in reactive astrocytes
Although abnormal mitosis with disarranged metaphase chromosomes or many micronuclei in astrocytes (named “Alzheimer I type astrocytes” and later “Creutzfeldt-Peters cells”) have been known for nearly 100 year...Acta Neuropathologica Communications 2020 8:47Insulin-like growth factor-1 overexpression increases long-term survival of posttrauma-born hippocampal neurons while inhibiting ectopic migration following traumatic brain injury
Cellular damage associated with traumatic brain injury (TBI) manifests in motor and cognitive dysfunction following injury. Experimental models of TBI reveal cell death in the granule cell layer (GCL) of the h...Acta Neuropathologica Communications 2020 8:46Biological links between traumatic brain injury and Parkinson’s disease
Parkinson’s Disease (PD) is a progressive neurodegenerative disorder with no cure. Clinical presentation is characterized by postural instability, resting tremors, and gait problems that result from progressiv...Acta Neuropathologica Communications 2020 8:45Tacr3 in the lateral habenula differentially regulates orofacial allodynia and anxiety-like behaviors in a mouse model of trigeminal neuralgia
Trigeminal neuralgia (TN) is debilitating and is usually accompanied by mood disorders. The lateral habenula (LHb) is considered to be involved in the modulation of pain and mood disorders, and the present stu...Acta Neuropathologica Communications 2020 8:44Diversity in Aβ deposit morphology and secondary proteome insolubility across models of Alzheimer-type amyloidosis
A hallmark pathology of Alzheimer’s disease (AD) is the formation of amyloid β (Aβ) deposits that exhibit diverse localization and morphologies, ranging from diffuse to cored-neuritic deposits in brain parench...Acta Neuropathologica Communications 2020 8:43Annexin A2–STAT3–Oncostatin M receptor axis drives phenotypic and mesenchymal changes in glioblastoma
Glioblastoma (GBM) is characterized by extensive tumor cell invasion, angiogenesis, and proliferation. We previously established subclones of GBM cells with distinct invasive phenotypes and identified annexin ...Acta Neuropathologica Communications 2020 8:42KLF4K409Q–mutated meningiomas show enhanced hypoxia signaling and respond to mTORC1 inhibitor treatment
Meningioma represents the most common primary brain tumor in adults. Recently several non-NF2 mutations in meningioma have been identified and correlated with certain pathological subtypes, locations and clinical...Acta Neuropathologica Communications 2020 8:41The molecular characteristics of spinal cord gliomas with or without H3 K27M mutation
Due to the rare incidence of spinal cord astrocytomas, their molecular features remain unclear. Here, we characterized the landscapes of mutations in H3 K27M, isocitrate dehydrogenase 1 (IDH1) R132H, BRAF V600E, ...Acta Neuropathologica Communications 2020 8:40Neuropathological correlates of parkinsonian disorders in a large Dutch autopsy series
The clinical diagnosis in patients with parkinsonian disorders can be challenging, and a definite diagnosis requires neuropathological confirmation. The aim of this study was to examine whether a clinical diag...Acta Neuropathologica Communications 2020 8:39Severe hydroxymethylbilane synthase deficiency causes depression-like behavior and mitochondrial dysfunction in a mouse model of homozygous dominant acute intermittent porphyria
Acute intermittent porphyria (AIP) is an autosomal dominant inborn error of heme biosynthesis due to a pathogenic mutation in the Hmbs gene, resulting in half-normal activity of hydroxymethylbilane synthase. Fact...Acta Neuropathologica Communications 2020 8:38- Acta Neuropathologica Communications 2020 8:37
Astrocytes rescue neuronal health after cisplatin treatment through mitochondrial transfer
Neurodegenerative disorders, including chemotherapy-induced cognitive impairment, are associated with neuronal mitochondrial dysfunction. Cisplatin, a commonly used chemotherapeutic, induces neuronal mitochond...Acta Neuropathologica Communications 2020 8:36Altered secretory and neuroprotective function of the choroid plexus in progressive multiple sclerosis
The choroid plexus (CP) is a key regulator of the central nervous system (CNS) homeostasis through its secretory, immunological and barrier properties. Accumulating evidence suggests that the CP plays a pivota...Acta Neuropathologica Communications 2020 8:35Loss of BICD2 in muscle drives motor neuron loss in a developmental form of spinal muscular atrophy
Autosomal dominant missense mutations in BICD2 cause Spinal Muscular Atrophy Lower Extremity Predominant 2 (SMALED2), a developmental disease of motor neurons. BICD2 is a key component of the cytoplasmic dynein/d...Acta Neuropathologica Communications 2020 8:34Histological correlates of postmortem ultra-high-resolution single-section MRI in cortical cerebral microinfarcts
The identification of cerebral microinfarctions with magnetic resonance imaging (MRI) and histological methods remains challenging in aging and dementia. Here, we matched pathological changes in the microvascu...Acta Neuropathologica Communications 2020 8:33CMT2Q-causing mutation in the Dhtkd1 gene lead to sensory defects, mitochondrial accumulation and altered metabolism in a knock-in mouse model
Charcot-Marie-Tooth disease (CMT) is a group of inherited neurological disorders of the peripheral nervous system. CMT is subdivided into two main types: a demyelinating form, known as CMT1, and an axonal form...Acta Neuropathologica Communications 2020 8:32
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