Analysis of inflammatory markers and tau deposits in an autopsy series of nine patients with anti-IgLON5 disease
AbstractAnti-IgLON5 disease is a rare neurological, probably autoimmune, disorder associated in many cases with a specific tauopathy. Only a few post-mortem neuropathological studies have been reported so far. Little is known about the pathogenic mechanisms that result in neurodegeneration. We investigated the neuropathology of anti-IgLON5 disease and characterized cellular and humoral inflammation. We included nine cases (six of them previously published). Median age of patients was 71 years (53–82 years), the median disease duration was 6 years (0.5–13 years), and the female to male ratio was 5:4. Six cases with...
Source: Acta Neuropathologica - October 1, 2023 Category: Neurology Source Type: research
Neuronal tau pathology worsens late-phase white matter degeneration after traumatic brain injury in transgenic mice
Conclusions: Neuronal tau pathology differentially exacerbated CC pathology based on i njury severity and chronicity. Ongoing CC atrophy from s-TBI became accompanied by late demyelination. Pathological tau significantly worsened CC atrophy during the chronic phase after r-mTBI. (Source: Acta Neuropathologica)
Source: Acta Neuropathologica - October 1, 2023 Category: Neurology Source Type: research
TDP-43 differentially propagates to induce antero- and retrograde degeneration in the corticospinal circuits in mouse focal ALS models
AbstractAmyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by TDP-43 inclusions in the cortical and spinal motor neurons. It remains unknown whether and how pathogenic TDP-43 spreads across neural connections to progress degenerative processes in the cortico-spinal motor circuitry. Here we established novel mouse ALS models that initially induced mutant TDP-43 inclusions in specific neuronal or cell types in the motor circuits, and investigated whether TDP-43 and relevant pathological processes spread across neuronal or cellular connections. We first developed ALS models that prima...
Source: Acta Neuropathologica - October 1, 2023 Category: Neurology Source Type: research
The variance in phosphorylated, insoluble ⍺-synuclein in humans, rats, and mice is not mainly driven by biological sex
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - October 1, 2023 Category: Neurology Source Type: research
Decreased dystrophin expression and elevated dystrophin-targeting miRNAs in anti-HMGCR immune-mediated necrotizing myopathy
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - October 1, 2023 Category: Neurology Source Type: research
Spatial immune profiling of glioblastoma identifies an inflammatory, perivascular phenotype associated with longer survival
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - October 1, 2023 Category: Neurology Source Type: research
Correction: APOE and TREM2 regulate amyloid-responsive microglia in Alzheimer ’s disease
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - October 1, 2023 Category: Neurology Source Type: research
Correction to: Intraneuronal aggregation of the β‑CTF fragment of APP (C99) induces Aβ‑independent lysosomal-autophagic pathology
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - October 1, 2023 Category: Neurology Source Type: research
Genetical and epigenetical profiling identifies two subgroups of pineal parenchymal tumors of intermediate differentiation (PPTID) with distinct molecular, histological and clinical characteristics
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - September 30, 2023 Category: Neurology Source Type: research
Senescent fibro-adipogenic progenitors are potential drivers of pathology in inclusion body myositis
In this study, we focused on senescence of tissue-resident cells as potential driver of disease. For this purpose, we compared IBM patients to non-diseased controls and immune-mediated necrotizing myopathy patients. Histopathological analysis suggested that cellular senescence is a prominent feature of IBM, primarily affecting non-myogenic cells. In-depth analysis by single nuclei RNA sequencing allowed for the deconvolution and study of muscle-resident cell populations. Among these, we identified a specific cluster of fibro-adipogenic progenitors (FAPs) that demonstrated key hallmarks of senescence, including a pro-inflam...
Source: Acta Neuropathologica - September 29, 2023 Category: Neurology Source Type: research
Normal meninges harbor oncogenic somatic mutations in meningioma-driver genes
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - September 26, 2023 Category: Neurology Source Type: research
Transcriptional mutagenesis of α-synuclein caused by DNA oxidation in Parkinson’s disease pathogenesis
In this study, we hypothesized that 8-oxodG can generate novel α-synuclein (α-SYN) mutant s with altered pathologic aggregation through a phenomenon called transcriptional mutagenesis (TM). We observed a significantly higher accumulation of 8-oxodG in the midbrain genomic DNA from PD patients compared to age-matched controls, both globally and region specifically to α-SYN. In-silico ana lysis predicted that forty-three amino acid positions can contribute to TM-derived α-SYN mutation. Here, we report a significantly higher load of TM-derived α-SYN mutants from the midbrain of PD patients compared to controls using a se...
Source: Acta Neuropathologica - September 23, 2023 Category: Neurology Source Type: research
Reply: Soluble oligomers or insoluble fibrils?
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - September 21, 2023 Category: Neurology Source Type: research
Soluble oligomers or insoluble fibrils? Scientific commentary on “Tau seeding and spreading in vivo is supported by both AD-derived fibrillar and oligomeric tau”
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - September 21, 2023 Category: Neurology Source Type: research
Co-pathology may impact outcomes of amyloid-targeting treatments: clinicopathological results from two patients treated with aducanumab
(Source: Acta Neuropathologica)
Source: Acta Neuropathologica - September 19, 2023 Category: Neurology Source Type: research
