Journal of Neuropathology and Experimental Neurology最新文献_第5页

Compound muscle action potential as an early functional in vivo measure of Sarm1 inhibition after sciatic nerve transection. 复合肌动作电位作为坐骨神经横断后Sarm1抑制的早期功能体内测量。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-06-06 DOI: 10.1093/jnen/nlaf066

Seong Kwon Hur, Rebecca R Leahey, Mitchell Geringer, Chang Hoon Cho, Hilda Hernandez-Barry, Jichu Pang, William S Sawyer, Miriam Baca, Marika Nespi, Raymond R Asuncion, ManKin Choy, James Maksymetz, Stephen T Vito, Jose Imperio, Kimberly Stark, Samantha A Green, Bryan K Chan, Luke Xie, Justin Ly, Alicia A Nugent, Jeffrey W Hofmann, Flora I Hinz, Martin Weber

{"title":"Compound muscle action potential as an early functional in vivo measure of Sarm1 inhibition after sciatic nerve transection.","authors":"Seong Kwon Hur, Rebecca R Leahey, Mitchell Geringer, Chang Hoon Cho, Hilda Hernandez-Barry, Jichu Pang, William S Sawyer, Miriam Baca, Marika Nespi, Raymond R Asuncion, ManKin Choy, James Maksymetz, Stephen T Vito, Jose Imperio, Kimberly Stark, Samantha A Green, Bryan K Chan, Luke Xie, Justin Ly, Alicia A Nugent, Jeffrey W Hofmann, Flora I Hinz, Martin Weber","doi":"10.1093/jnen/nlaf066","DOIUrl":"https://doi.org/10.1093/jnen/nlaf066","url":null,"abstract":"The NADase sterile alpha and TIR motif containing 1 (Sarm1) protein drives axon degeneration after injury. Loss or inhibition of Sarm1 structurally protects axons after sciatic nerve transection (SNT) in vivo but whether Sarm1 also drives functional loss after nerve injury is less clear. We established compound muscle action potential (CMAP) as a novel functional correlate of Sarm1 activation in a SNT mouse model and evaluated its relationship with biochemical and a novel Cellpose-based histological axon detection measure. CMAP amplitudes were elicited 8 h post-SNT but reached near-floor levels by 24 h. Decreases in CMAP amplitude are delayed in a gene dose-dependent manner in Sarm1 knockout mice or by pharmacological Sarm1 inhibition. Myelinated axon density, the NAD hydrolysis product cyclic adenosine diphosphate ribose (cADPR), and the axon degeneration plasma biomarker neurofilament light (NfL) were all altered in a Sarm1-dependent manner. In wild type mice, axon density and NfL were altered at time points after that of cADPR and functional loss, indicating that functional deficits preceded structural deficits. We conclude that functional and structural declines after injury are delayed by Sarm1 inhibition and that CMAP measures after SNT can serve as a novel, preclinical, functional, pharmacodynamic readout for Sarm1 inhibition.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144234396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

PAS+/PASD- intracytoplasmic glycogen is not specific for clear cell meningioma. PAS+/PASD-胞浆内糖原对透明细胞脑膜瘤无特异性。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-06-06 DOI: 10.1093/jnen/nlaf065

Merryl Terry, Gerald Reis, Melike Pekmezci, Joanna J Phillips, Andrew Bollen, Tarik Tihan, Arie Perry

引用次数: 0

Alzheimer disease-associated tau post-translational modification mimics impact tau propagation and uptake. 阿尔茨海默病相关的tau翻译后修饰模拟影响tau的繁殖和摄取。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-06-01 DOI: 10.1093/jnen/nlaf007

John R Dickson, Robert G R Sobolewski, Analiese R Fernandes, Joanna M Cooper, Zhanyun Fan, Mirra Chung, Cameron Donahue, Derek H Oakley, Dudley K Strickland, Bradley T Hyman

{"title":"Alzheimer disease-associated tau post-translational modification mimics impact tau propagation and uptake.","authors":"John R Dickson, Robert G R Sobolewski, Analiese R Fernandes, Joanna M Cooper, Zhanyun Fan, Mirra Chung, Cameron Donahue, Derek H Oakley, Dudley K Strickland, Bradley T Hyman","doi":"10.1093/jnen/nlaf007","DOIUrl":"10.1093/jnen/nlaf007","url":null,"abstract":"As Alzheimer disease (AD) progresses, pathological tau spreads by cell-to-cell propagation of tau. This study aims to elucidate the impact of AD-associated post-translational modifications of tau-on-tau propagation. Tau propagation reporter constructs distinguishing donor cells from recipient cells were developed, and additional constructs were made with tau residues mutated from serine or threonine to aspartate to mimic the negative charge of a phosphorylation and/or from lysine to glutamine to mimic the charge-neutralizing effect of acetylation. Flow cytometry was used to quantify donor and recipient cells. This revealed that the mutations generally tended to reduce tau propagation compared to wildtype tau. Recombinant tau containing either wildtype or posttranslational modification mimicking mutations were used to treat Chinese hamster ovary cells or human induced pluripotent stem cell-derived neurons to quantify tau uptake, revealing that the mutations generally resulted in reduced uptake compared to wildtype tau. Surface plasmon resonance revealed that the mutations had a reduced affinity for lipoprotein receptor-related protein 1 (LRP1), a tau uptake receptor, compared to wildtype tau. Overall, these results suggest that AD-associated posttranslational modification mimicking mutations reduce the cell-to-cell propagation of tau by reducing tau uptake by recipient cells, which may be in part due to reduced binding affinity to LRP1.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":"459-470"},"PeriodicalIF":3.2,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12096005/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143472566","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Shift of DNA methylation class may occur on recurrence of pleomorphic xanthoastrocytoma. 多形性黄色星形细胞瘤复发时可能发生DNA甲基化类别的转移。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-27 DOI: 10.1093/jnen/nlaf061

Gonzalo Hernandez Gamero, Evelina Miele, Andrea Mafficini, Lucrezia Ghione, Sara Patrizi, Lucia Pedace, Serena Ammendola, Valeria Barresi

引用次数: 0

Metastases to dysplastic cerebellar gangliocytomas. 转移到发育不良的小脑神经节细胞瘤。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-20 DOI: 10.1093/jnen/nlaf054

Sara Hayama, Chase Hwang, Christopher Lum, Jane Uyehara-Lock, Wichit Sae-Ow, Koah Vierkoetter, Brock Kaya

引用次数: 0

Multi-omic analysis of meningeal cerebral amyloid angiopathy reveals enrichment of unsubstituted glucosamine and extracellular proteins. 脑膜脑淀粉样血管病的多组学分析显示未取代的氨基葡萄糖和细胞外蛋白富集。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-01 DOI: 10.1093/jnen/nlaf018

Joshua E Mayfield, Alexander J Rajic, Patricia Aguilar-Calvo, Katrin Soldau, Samantha Flores, Roger Lawrence, Biwsa Choudhury, Majid Ghassemian, Donald P Pizzo, Steven L Wagner, Garrett A Danque, Paige Sumowski, Lawrence A Hansen, Vanessa Goodwill, Jeffery D Esko, Christina J Sigurdson

{"title":"Multi-omic analysis of meningeal cerebral amyloid angiopathy reveals enrichment of unsubstituted glucosamine and extracellular proteins.","authors":"Joshua E Mayfield, Alexander J Rajic, Patricia Aguilar-Calvo, Katrin Soldau, Samantha Flores, Roger Lawrence, Biwsa Choudhury, Majid Ghassemian, Donald P Pizzo, Steven L Wagner, Garrett A Danque, Paige Sumowski, Lawrence A Hansen, Vanessa Goodwill, Jeffery D Esko, Christina J Sigurdson","doi":"10.1093/jnen/nlaf018","DOIUrl":"10.1093/jnen/nlaf018","url":null,"abstract":"Cerebral amyloid angiopathy (CAA) is a common feature of Alzheimer's disease in which amyloid-β (Aβ) deposits in cerebral and leptomeningeal vessel walls, predisposing vessels to micro- and macro-hemorrhages. The vessel walls contain distinct proteins and heparan sulfate (HS), yet how vascular proteins and HS jointly associate with Aβ is unknown. We conducted the first multi-omics study to systematically characterize the proteins as well as the HS abundance, sulfation level, and disaccharide composition of leptomeninges from 23 moderate to severe CAA cases and controls. We then analyzed the associations between Aβ and other proteins, HS, and apolipoprotein E genotype. We found an increase in a minor HS disaccharide containing unsubstituted glucosamine, as well as 6-O sulfated disaccharides; Aβ40 levels positively correlated with unsubstituted glucosamine. There was also an increase in extracellular proteins derived from brain parenchyma or plasma, including olfactomedin-like protein 3, fibrinogen, serum amyloid protein, apolipoprotein E, and secreted frizzled related protein-3. Our findings of vascular HS and protein alterations specific to CAA-affected leptomeningeal vessels provide molecular insight into the extracellular remodeling that co-occurs with Aβ deposits and may indicate a basis for antemortem diagnostic assay development and therapeutic strategies to impede Aβ-HS interactions.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":"398-411"},"PeriodicalIF":3.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12012350/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143743236","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Solitary fibrous tumor in the pineal region: A series of 5 cases and literature review. 松果体区孤立性纤维性肿瘤：附5例报告并文献复习。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-01 DOI: 10.1093/jnen/nlaf008

Shannon M Robins, Mary Rosenblatt, Jeffrey N Bruce, Peter Canoll, George Zanazzi

{"title":"Solitary fibrous tumor in the pineal region: A series of 5 cases and literature review.","authors":"Shannon M Robins, Mary Rosenblatt, Jeffrey N Bruce, Peter Canoll, George Zanazzi","doi":"10.1093/jnen/nlaf008","DOIUrl":"10.1093/jnen/nlaf008","url":null,"abstract":"Solitary fibrous tumors (SFTs) are fibroblastic mesenchymal neoplasms defined by the presence of a NAB2::STAT6 fusion and exhibit a broad range of behaviors. SFTs in the pineal region are poorly understood due to the limited number of reported cases. Here, we report a 48-year-old woman with a pineal region SFT who subsequently developed metastatic left para-falcine parieto-occipital and right lung upper lobe SFTs over the next 12 years. This was the only pineal SFT identified in an institutional cohort of 34 resected pineal region lesions. Review of another, much larger institutional cohort of pineal region lesions revealed 4 additional patients with SFT but none with extracranial metastasis. We present descriptions of their clinical presentations, treatments, histopathologic findings, available genomic alterations, and longitudinal outcomes. Finally, we performed a comprehensive literature search and identified 19 individual patients with pineal region SFTs. None of these reported neoplasms had an extracranial metastasis. Taken together, this work contributes to the growing body of data characterizing this rare tumor with aggressive potential and reinforces SFTs as a possible differential diagnosis for pineal region tumors.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":"379-390"},"PeriodicalIF":3.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12012368/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143414613","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Correlating hippocampal and amygdala volumes with neuropathological burden in Down syndrome and Alzheimer's disease and related neurodegenerative pathologies using 7T postmortem MRI. 使用7T死后MRI分析唐氏综合征和阿尔茨海默病及相关神经退行性病理的海马和杏仁核体积与神经病理负担的关系

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-01 DOI: 10.1093/jnen/nlaf010

Jr-Jiun Liou, Jinghang Li, Jacob Berardinelli, Hecheng Jin, Tales Santini, Jaehoon Noh, Nadim Farhat, Minjie Wu, Howard J Aizenstein, Joseph M Mettenburg, William H Yong, Elizabeth Head, Milos D Ikonomovic, Tamer S Ibrahim, Julia K Kofler

{"title":"Correlating hippocampal and amygdala volumes with neuropathological burden in Down syndrome and Alzheimer's disease and related neurodegenerative pathologies using 7T postmortem MRI.","authors":"Jr-Jiun Liou, Jinghang Li, Jacob Berardinelli, Hecheng Jin, Tales Santini, Jaehoon Noh, Nadim Farhat, Minjie Wu, Howard J Aizenstein, Joseph M Mettenburg, William H Yong, Elizabeth Head, Milos D Ikonomovic, Tamer S Ibrahim, Julia K Kofler","doi":"10.1093/jnen/nlaf010","DOIUrl":"10.1093/jnen/nlaf010","url":null,"abstract":"Limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC), is common in elderly brains and often seen in conjunction with Alzheimer's disease neuropathologic change (ADNC). LATE-NC typically begins in the amygdala and spreads to the hippocampus and neocortex. Whether it contributes to hippocampal and amygdala atrophy in Down syndrome (DS) remains unexplored. We analyzed amygdala and hippocampal volumes and neuropathological burden in 12 DS cases and 54 non-DS cases with AD and related neurodegenerative pathologies (ADRNP) using 7 Tesla (7T) postmortem ex vivo MRI. Postmortem and antemortem hippocampal volumes were significantly correlated in a subset of 17 cases with available antemortem MRI scans. DS cases had smaller hippocampal and amygdala volumes than ADRNP cases; these correlated with more severe Braak stage but not with Thal phase. LATE-NC and hippocampal sclerosis (HS) were uncommon in DS cases. In ADRNP cases, lower hippocampal volumes associated with dementia duration, advanced Thal phase, Braak NFT stage, C score, LATE-NC stage, HS and arteriolosclerosis severity; reduced amygdala volumes correlated with severe LATE-NC stage, HS, and arteriolosclerosis severity, but not with Thal phase or Braak NFT stage. Lewy body pathology did not affect hippocampal or amygdala volume in either cohort. Thus, hippocampal volumes in ADRNP were influenced by both ADNC and LATE-NC, and amygdala volumes were primarily influenced by LATE-NC. In DS, hippocampal and amygdala volumes were primarily influenced by tau pathology.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":"364-378"},"PeriodicalIF":3.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12012357/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143597038","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Cylindrical spirals in a patient with fascioscapulohumeral muscular dystrophy. 筋膜肩肱肌营养不良患者的圆柱形螺旋。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-01 DOI: 10.1093/jnen/nlae133

Qiang Gang, Jing Chen, Meng Yu, Zhaoxia Wang, Yun Yuan, Wei Zhang

引用次数: 0

Re: Article by Machaalani et al. concerning dentate gyrus dysplasia. 回复：Machaalani等人关于齿状回发育不良的文章。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-01 DOI: 10.1093/jnen/nlaf011

Douglas C Miller

引用次数: 0