Journal of experimental neurology最新文献_第6页

Retroviral Elements in Human Evolution and Neural Development. 逆转录病毒在人类进化和神经发育中的作用。

Journal of experimental neurology Pub Date : 2021-01-01

Tongguang Wang, Tara T Doucet-O'Hare, Lisa Henderson, Rachel P M Abrams, Avindra Nath

引用次数: 0

The Spread of Spectrin in Ataxia and Neurodegenerative Disease. 幽灵蛋白在共济失调和神经退行性疾病中的传播。

Journal of experimental neurology Pub Date : 2021-01-01

Jon S Morrow, Michael C Stankewich

{"title":"The Spread of Spectrin in Ataxia and Neurodegenerative Disease.","authors":"Jon S Morrow, Michael C Stankewich","doi":"","DOIUrl":"","url":null,"abstract":"Experimental and hereditary defects in the ubiquitous scaffolding proteins of the spectrin gene family cause an array of neuropathologies. Most recognized are ataxias caused by missense, deletions, or truncations in the SPTBN2 gene that encodes beta III spectrin. Such mutations disrupt the organization of post-synaptic receptors, their active transport through the secretory pathway, and the organization and dynamics of the actin-based neuronal skeleton. Similar mutations in SPTAN1 that encodes alpha II spectrin cause severe and usually lethal neurodevelopmental defects including one form of early infantile epileptic encephalopathy type 5 (West syndrome). Defects in these and other spectrins are implicated in degenerative and psychiatric conditions. In recent published work, we describe in mice a novel variant of alpha II spectrin that results in a progressive ataxia with widespread neurodegenerative change. The action of this variant is distinct, in that rather than disrupting a constitutive ligand-binding function of spectrin, the mutation alters its response to calcium and calmodulin-regulated signaling pathways including its response to calpain activation. As such, it represents a novel spectrinopathy that targets a key regulatory pathway where calcium and tyrosine kinase signals converge. Here we briefly discuss the various roles of spectrin in neuronal processes and calcium activated regulatory inputs that control its participation in neuronal growth, organization, and remodeling. We hypothesize that damage to the neuronal spectrin scaffold may be a common final pathway in many neurodegenerative disorders. Targeting the pathways that regulate spectrin function may thus offer novel avenues for therapeutic intervention.","PeriodicalId":73744,"journal":{"name":"Journal of experimental neurology","volume":"2 3","pages":"131-139"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8439443/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39423195","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Differential Fecal Microbiome Dysbiosis after Equivalent Traumatic Brain Injury in Aged Versus Young Adult Mice. 老年和年轻成年小鼠等效创伤性脑损伤后粪便微生物组的差异性失调。

Journal of experimental neurology Pub Date : 2021-01-01 DOI: 10.33696/neurol.2.044

Booker T Davis, Mecca B A R Islam, Promi Das, Jack A Gilbert, Karen J Ho, Steven J Schwulst

{"title":"Differential Fecal Microbiome Dysbiosis after Equivalent Traumatic Brain Injury in Aged Versus Young Adult Mice.","authors":"Booker T Davis, Mecca B A R Islam, Promi Das, Jack A Gilbert, Karen J Ho, Steven J Schwulst","doi":"10.33696/neurol.2.044","DOIUrl":"10.33696/neurol.2.044","url":null,"abstract":"Traumatic brain injury (TBI) has a bimodal age distribution with peak incidence at age 24 and age 65 with worse outcomes developing in aged populations. Few studies have specifically addressed age at the time of injury as an independent biologic variable in TBI-associated secondary pathology. Within the framework of our published work, identifying age related effects of TBI on neuropathology, cognition, memory and motor function we analyzed fecal pellets collected from young and aged TBI animals to assess for age-induced effects in TBI induced dysbiosis. In this follow up, work we hypothesized increased dysbiosis after TBI in aged (80-week-old, N=10) versus young (14-week-old, N=10) mice. C57BL/6 males received a sham incision or TBI via open-head controlled cortical impact. Fresh stool pellets were collected 1-day pre-TBI, then 1, 7, and 28-days post-TBI for 16S rRNA gene sequencing and taxonomic analysis. Data revealed an age induced increase in disease associated microbial species which were exacerbated by injury. Consistent with our hypothesis, aged mice demonstrated a high number of disease associated changes to the gut microbiome pre- and post-injury. Our data suggest divergent microbiome phenotypes in injury between young and aged reflecting a previously unknown interaction between age, TBI, and the gut-brain axis implying the need for different treatment strategies.","PeriodicalId":73744,"journal":{"name":"Journal of experimental neurology","volume":"2 3","pages":"120-130"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8612634/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39660057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4

Capillary Stalling: A Mechanism of Decreased Cerebral Blood Flow in AD/ADRD. 毛细血管停滞：AD/ADRD脑血流量减少的机制。

Journal of experimental neurology Pub Date : 2021-01-01 DOI: 10.33696/neurol.2.048

Reece Crumpler, Richard J Roman, Fan Fan

{"title":"Capillary Stalling: A Mechanism of Decreased Cerebral Blood Flow in AD/ADRD.","authors":"Reece Crumpler, Richard J Roman, Fan Fan","doi":"10.33696/neurol.2.048","DOIUrl":"10.33696/neurol.2.048","url":null,"abstract":"Alzheimer's Disease (AD) and Alzheimer's Disease-Related Dementias (ADRD) are debilitating conditions that are highly associated with aging populations, especially those with comorbidities such as diabetes and hypertension. In addition to the classical pathological findings of AD, such as beta-amyloid (Aβ) accumulation and tau hyperphosphorylation, vascular dysfunction is also associated with the progression of the disease. Vascular dysfunction in AD is associated with decreased cerebral blood flow (CBF). Impaired CBF is an early and persistent symptom of AD/ADRD and is thought to be associated with deficient autoregulation and neurovascular coupling. Another recently elucidated mechanism that contributes to cerebral hypoperfusion is capillary stalling, or the temporary arrest of capillary blood flow usually precipitated by a stalled leukocyte or constriction of actin-containing capillary pericytes. Stalled capillaries are associated with decreased CBF and impaired cognitive performance. AD/ADRD are associated with chronic, low-level inflammation, which contributes to capillary stalling by increased cell adhesion molecules, circulating leukocytes, and reactive oxygen species production. Recent research has shed light on potential targets to decrease capillary stalling in AD mice. Separate inhibition of Ly6G and VEGF-A has been shown to decrease capillary stalling and increase CBF in AD mice. These results suggest that targeting stalled capillaries could influence the outcome of AD and potentially be a target for future therapies.","PeriodicalId":73744,"journal":{"name":"Journal of experimental neurology","volume":"2 4","pages":"149-153"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8754422/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39910127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Targeting the Cancer Stem Cell (CSC) Phenotype: Uprooting the Evil Seed 靶向癌症干细胞(CSC)表型:根除邪恶的种子

Journal of experimental neurology Pub Date : 2020-12-31 DOI: 10.33696/NEUROL.1.027

H. Lopez-Bertoni, J. Laterra

引用次数: 0

Alzheimer and It’s Possible Therapy: A Review 阿尔茨海默病及其可能的治疗方法综述

Journal of experimental neurology Pub Date : 2020-12-31 DOI: 10.33696/NEUROL.1.019

A. Chakraborty, Anil Diwan

引用次数: 1

Cutaneous Side Effects of First-Second Line Oral Disease - Modifying Treatments in Patients with Multiple Sclero 多发性硬化症患者一二线口腔疾病的皮肤副作用-改良治疗

Journal of experimental neurology Pub Date : 2020-12-31 DOI: 10.33696/NEUROL.1.024

Doruk Arslan, A. Tuncer

引用次数: 0

Can ECT Improve the Motor Symptoms of a Neurological Disease? A Case of Dopa-Responsive Dystonia ECT能改善神经系统疾病的运动症状吗？多巴反应性肌张力障碍1例

Journal of experimental neurology Pub Date : 2020-12-31 DOI: 10.33696/NEUROL.1.023

C. Guillet

引用次数: 0

A Commentary on Concomitant Symptomatic Coronary Disease and Carotid Artery Stenosis -The Tufts Medical Center Experience 对伴随症状性冠状动脉疾病和颈动脉狭窄的评论-塔夫茨医疗中心的经验

Journal of experimental neurology Pub Date : 2020-12-31 DOI: 10.33696//NEUROL.1.021

Y. Ikeno, K. Charlton-Ouw, M. Iafrati, Anand Y. Shah

{"title":"A Commentary on Concomitant Symptomatic Coronary Disease and Carotid Artery Stenosis -The Tufts Medical Center Experience","authors":"Y. Ikeno, K. Charlton-Ouw, M. Iafrati, Anand Y. Shah","doi":"10.33696//NEUROL.1.021","DOIUrl":"https://doi.org/10.33696//NEUROL.1.021","url":null,"abstract":"135 Despite the development of surgical outcomes, acute stroke remain a devastating complication following coronary artery bypass grafting (CABG) [1]. Coexistent CAD and carotid artery stenosis are prevalent due to their common background of systemic atherosclerosis (20%) [2]. Naylor, et al. [3,4] demonstrated that the risk of stroke associated with CABG is 3.2% in patients with asymptomatic, unilateral severe carotid stenosis, whereas these figures increase to 5.2% in those with bilateral carotid stenosis and 7% to 11.6% in those with carotid occlusion. The management of concomitant CAD and carotid artery disease has been elucidated over time. The combination of carotid endarterectomy (CEA) and CABG in the same surgical setting was introduced in the 1980s [5]. Nonetheless, the surgical management, particularly the timing and order of surgical procedures, remains varied across North America. This commentary reflects upon the Tufts Medical Center experience on the current knowledge of the prevention of perioperative stroke in patients with concurrent CAD and carotid artery disease, focusing on simultaneous CEA/CABG.","PeriodicalId":73744,"journal":{"name":"Journal of experimental neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45625841","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

A Protocol for the Generation of Treatment-naïve Biopsyderived Diffuse Intrinsic Pontine Glioma and Diffuse Midline Glioma Models 一种生成治疗幼稚活检衍生的弥漫性固有脑桥胶质瘤和弥漫性中线胶质瘤模型的方案

Journal of experimental neurology Pub Date : 2020-12-31 DOI: 10.33696/neurol.1.025

{"title":"A Protocol for the Generation of Treatment-naïve Biopsyderived Diffuse Intrinsic Pontine Glioma and Diffuse Midline Glioma Models","authors":"","doi":"10.33696/neurol.1.025","DOIUrl":"https://doi.org/10.33696/neurol.1.025","url":null,"abstract":"Diffuse intrinsic pontine glioma (DIPG) is a universally fatal tumor of the brainstem, most commonly affecting young children. Due to its location, surgical resection is not achievable, but consideration of a biopsy has become standard practice at children’s hospitals with the appropriate neurosurgical expertise. While the decision to obtain a biopsy should be directed by the presence of atypical radiographic features that call the diagnosis of DIPG into question or the requirement of biopsy tissue for clinical trial enrollment, once this precious tissue is available its use for research should be considered. The majority of DIPG and diffuse midline glioma, H3 K27Mmutant (DMG) models are autopsy-derived or genetically-engineered, each of which has limitations for translational studies, so the use of biopsy tissue for laboratory model development provides an opportunity to create unique model systems. Here, we present a detailed laboratory protocol for the generation of treatment-naïve biopsy-derived DIPG/DMG models. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. http://creativecommons.org/ licenses/by/4.0/ Correspondence should be addressed to Nicholas A. Vitanza; nicholas.vitanza@seattlechildrens.org. Authorship MCB, AN, CM, SMM, CAW, FP, JMO, and NAV participated in the design or interpretation of the reported experiments or results. MCB, AN, CM, SMM, CAW, FP, BLC, SRB, and NAV participated in the acquisition or analysis of data. MCB, AN, and NAV wrote the manuscript with revisions and approval from all authors. NAV supervised all aspects of the research. Conflicts of Interest None. HHS Public Access Author manuscript J Exp Neurol. Author manuscript; available in PMC 2021 March 24. Published in final edited form as: J Exp Neurol. 2020 December ; 1(4): 158–167. doi:10.33696//Neurol.1.025. A uhor M anscript","PeriodicalId":73744,"journal":{"name":"Journal of experimental neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42468937","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1