Journal of Neuropathology and Experimental Neurology最新文献_第3页

Aggressive behavior in a molecularly benign tumor: The diagnostic odyssey of a pediatric pilocytic astrocytoma with initial high-grade histologic features and rapid recurrence. 分子良性肿瘤的侵袭行为：儿童毛细胞星形细胞瘤的诊断历程，其最初的高级别组织学特征和快速复发。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-22 DOI: 10.1093/jnen/nlaf055

Bilge Yaşar, Bahattin Tanrıkulu, Ayça Erşen Danyeli, M Memet Özek

引用次数: 0

Phospholipase C epsilon 1 aggravates β-amyloid-associated cognitive impairments and pyroptosis through activating the RAC1-STAT3 pathway in Alzheimer disease models. 在阿尔茨海默病模型中，磷脂酶C ε 1通过激活RAC1-STAT3通路加重β-淀粉样蛋白相关的认知障碍和焦亡。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-22 DOI: 10.1093/jnen/nlaf048

Lijuan Pei, Wenjuan Hu, Li Wang, Hongbin Cai

{"title":"Phospholipase C epsilon 1 aggravates β-amyloid-associated cognitive impairments and pyroptosis through activating the RAC1-STAT3 pathway in Alzheimer disease models.","authors":"Lijuan Pei, Wenjuan Hu, Li Wang, Hongbin Cai","doi":"10.1093/jnen/nlaf048","DOIUrl":"https://doi.org/10.1093/jnen/nlaf048","url":null,"abstract":"Increased phospholipase C epsilon 1 (PLCE1) gene expression has been observed in patients with Alzheimer disease (AD), but its roles in AD remain unclear. This study aimed to explore the effects of PLCE1 expression in AD models. Rats were divided into 4 groups: sham+AAV-shNC (negative control), sham+AAV-shPLCE1, Aβ+AAV-shNC, and Aβ + AAV-shPLCE1. To investigate the effects of PLCE1 expression following β-amyloid administration, its expression was measured at both the mRNA and protein levels. Cognitive function was assessed using the Morris water maze. Immunofluorescence and Nissl staining of cerebral cortical tissues demonstrated that PLCE1 downregulation alleviated β-amyloid-induced brain injury. TUNEL, Western blot, and Enzyme-linked Immunosorbent Assay (ELISA) assays also showed that PLCE1 downregulation inhibited pyroptosis and inflammatory markers in the rats and in SH-SY5Y neuroblastoma cells. Using MTT, TUNEL, Western blot, and ELISA assays, we found that upregulation of the GTPase RAC1, which belongs to the RAS superfamily of small GTP-binding proteins, reversed the neuroprotective effect of PLCE1 downregulation. Together, this study suggests that PLCE1 may worsen AD by activating the STAT3 pathway through RAC1 upregulation. Overall, PLCE1 may exacerbate β-amyloid-induced cognitive impairments and pyroptosis via the RAC1-STAT3 pathway in AD patients.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144127949","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

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

Biological role and clinicopathological significance of leucine-rich α-2 glycoprotein 1 in the glioblastoma microenvironment. 富亮氨酸α-2糖蛋白1在胶质母细胞瘤微环境中的生物学作用及临床病理意义。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-09 DOI: 10.1093/jnen/nlaf049

Takuya Furuta, Hiroaki Miyoshi, Mayuko Moritsubo, Riho Nakajima, Tetsuya Negoto, Hideo Nakamura, Motohiro Morioka, Yasuo Uchida, Sumio Ohtsuki, Mitsutoshi Nakada

{"title":"Biological role and clinicopathological significance of leucine-rich α-2 glycoprotein 1 in the glioblastoma microenvironment.","authors":"Takuya Furuta, Hiroaki Miyoshi, Mayuko Moritsubo, Riho Nakajima, Tetsuya Negoto, Hideo Nakamura, Motohiro Morioka, Yasuo Uchida, Sumio Ohtsuki, Mitsutoshi Nakada","doi":"10.1093/jnen/nlaf049","DOIUrl":"https://doi.org/10.1093/jnen/nlaf049","url":null,"abstract":"Pseudoprogression, often misinterpreted as glioblastoma progression on MRI, results from treatment-induced inflammation and can resolve without additional intervention. This study investigated the role of leucine-rich α-2 glycoprotein 1 (LRG1) in the glioblastoma microenvironment. Leucine-rich α-2 glycoprotein 1 is associated with inflammation and prognosis in various diseases and its blood concentrations reflect disease-related inflammation. We focused on LRG1 expression in reactive astrocytes and assessed its potential as a biomarker for glioblastoma pseudoprogression. Cases with high LRG1 expression exhibited a distinct molecular profile, with increased angiogenesis-related gene expression and reduced stem cell-related gene activity, underscoring its dual role in tumor biology and progression. In vitro experiments demonstrated that LRG1 suppressed tumor cell invasion, supporting its inverse correlation with tumor cell stemness. Immunohistochemical analysis revealed that astrocytic LRG1 was associated with heightened peritumoral inflammation, characterized by CD8+ T-cell infiltration at the tumor periphery; this correlated with higher pseudoprogression rates and poorer prognosis. By providing a histopathological marker for pseudoprogression, LRG1 complements current imaging modalities and offers a novel approach to addressing unresolved diagnostic challenges in glioblastoma. These findings establish LRG1 as a promising biomarker that could aid clinicians in distinguishing pseudoprogression from true progression, ultimately enhancing personalized treatment strategies for glioblastoma patients.","PeriodicalId":16682,"journal":{"name":"Journal of Neuropathology and Experimental Neurology","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-05-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144026613","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

Reactive astrocytes in spinal cord injury: An analysis of heterogeneity based on temporality and spatiality, potential therapies, and limitations. 脊髓损伤中的反应性星形胶质细胞：基于时间和空间、潜在治疗和局限性的异质性分析。

IF 3.2 3区医学

Journal of Neuropathology and Experimental Neurology Pub Date : 2025-05-02 DOI: 10.1093/jnen/nlaf042

Mengting Qian, Zheng Wang, Hang Liu, Xinyu Zhang, Jingyi Xu, Yiwen Zhang, Ligang Chen, Zhengjun Zhou, Yang Yu, Wei Dong

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

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

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

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