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A Rationally Engineered Capsid Variant Of Aav9 For Systemic Cns-Directed And Peripheral Tissue-Detargeted Gene Delivery In Neonates, Dan Wang, Shaoyong Li, Dominic J. Gessler, Jun Xie, Li Zhong, Jia Li, Karen Tran, Kim Van Vliet, Lingzhi Ren, Qin Su, Ran He, Jason E. Goetzmann, Terence R. Flotte, Mavis Agbandje-McKenna, Guangping Gao 2018 University of Massachusetts Medical School

A Rationally Engineered Capsid Variant Of Aav9 For Systemic Cns-Directed And Peripheral Tissue-Detargeted Gene Delivery In Neonates, Dan Wang, Shaoyong Li, Dominic J. Gessler, Jun Xie, Li Zhong, Jia Li, Karen Tran, Kim Van Vliet, Lingzhi Ren, Qin Su, Ran He, Jason E. Goetzmann, Terence R. Flotte, Mavis Agbandje-Mckenna, Guangping Gao

Open Access Articles

Adeno-associated virus (AAV) has provided the gene therapy field with the most powerful in vivo gene delivery vector to realize safe, efficacious, and sustainable therapeutic gene expression. Because many clinically relevant properties of AAV-based vectors are governed by the capsid, much research effort has been devoted to the development of AAV capsids for desired features. Here, we combine AAV capsid discovery from nature and rational engineering to report an AAV9 capsid variant, designated as AAV9.HR, which retains AAV9's capability to traverse the blood-brain barrier and transduce neurons. This variant shows reduced transduction in peripheral tissues when delivered through ...


Ephrinb3 Modulates Hippocampal Neurogenesis And The Reelin Signaling Pathway In A Pilocarpineinduced Model Of Epilepsy, Tian-Tian Liu, Yi Li, Yi Shu, Bo Xiao, Li Feng 2018 Central South University

Ephrinb3 Modulates Hippocampal Neurogenesis And The Reelin Signaling Pathway In A Pilocarpineinduced Model Of Epilepsy, Tian-Tian Liu, Yi Li, Yi Shu, Bo Xiao, Li Feng

Open Access Articles

EphrinB3 is important in the regulation of cell proliferation, differentiation and migration via cellcell contact, and can activate the reelin pathway during brain development. However, the effect of ephrinB3 on hippocampal neurogenesis and the reelin pathway in epilepsy remains to be fully elucidated. In the present study, the expression of ephrinB3 in pilocarpineinduced status epilepticus (SE) rats was investigated. SYBR Greenbased reverse transcriptionquantitative polymerase chain reaction analysis, immunohistochemical labeling and western blot analysis were used to detect the gene and protein expression levels of ephrinB3 and reelin pathway proteins. Immunofluorescence staining of doublecortin (DCX) was utilized to analyze hippocampal neurogenesis ...


Magneto-Electric Nano-Particles For Non-Invasive Brain Stimulation, Kun Yue, Rakesh Guduru, Jeongmin Hong, Ping Liang, Madhavan Nair, Sakhrat Khizroev 2018 Center for Nanomedicine, College of Engineering and Computing, Florida International University, Miami, Florida

Magneto-Electric Nano-Particles For Non-Invasive Brain Stimulation, Kun Yue, Rakesh Guduru, Jeongmin Hong, Ping Liang, Madhavan Nair, Sakhrat Khizroev

Sakhrat Khizroev

This paper for the first time discusses a computational study of using magneto-electric (ME) nanoparticles to artificially stimulate the neural activity deep in the brain. The new technology provides a unique way to couple electric signals in the neural network to the magnetic dipoles in the nanoparticles with the purpose to enable a non-invasive approach. Simulations of the effect of ME nanoparticles for non-invasively stimulating the brain of a patient with Parkinson’s Disease to bring the pulsed sequences of the electric field to the levels comparable to those of healthy people show that the optimized values for the concentration ...


Role Of The Dorsal Striatum In Learning And Decision Making, Nole M. Hiebert 2018 The University of Western Ontario

Role Of The Dorsal Striatum In Learning And Decision Making, Nole M. Hiebert

Electronic Thesis and Dissertation Repository

The striatum, the input region of the basal ganglia, has been shown to mediate many cognitive functions. The striatum itself can be functionally segregated into dorsal (DS) and ventral striatum (VS). For more than 60 years, DS has been reported to mediate stimulus-response learning, though evidence has been accruing pointing to a role in decision making. These literatures have been growing independently and an aim of this thesis was to bridge these two bodies of knowledge. We directly investigated the role of DS in stimulus-response learning versus decision making using functional magnetic resonance imaging (fMRI) in patients with Parkinson’s ...


Spinal Cord Trauma: An Overview Of Normal Structure And Function, Primary And Secondary Mechanisms Of Injury, And Emerging Treatment Modalities, Daniel Morin 2018 Liberty University

Spinal Cord Trauma: An Overview Of Normal Structure And Function, Primary And Secondary Mechanisms Of Injury, And Emerging Treatment Modalities, Daniel Morin

Senior Honors Theses

The structures of the spinal cord and vertebral column are designed to provide flexibility, while still providing ample protection for the spinal cord deep within. While it does offer remarkable protection against most routine trauma, the spinal cord is still vulnerable to high-force etiologies of trauma and may become damaged as a result. These events are referred to as primary injury. Following the initial injury, the body’s own physiological responses cause a cascade of deleterious effects, known as secondary injury. Secondary injury is a major therapeutic target in mitigating the effects of spinal cord injury (SCI), and much research ...


Amygdala Corticofugal Input Shapes Mitral Cell Responses In The Accessory Olfactory Bulb, Livio Oboti, Eleonora Russo, Tuyen Tran, Daniel Durstewitz, Joshua G. Corbin 2018 George Washington University

Amygdala Corticofugal Input Shapes Mitral Cell Responses In The Accessory Olfactory Bulb, Livio Oboti, Eleonora Russo, Tuyen Tran, Daniel Durstewitz, Joshua G. Corbin

Pediatrics Faculty Publications

Interconnections between the olfactory bulb and the amygdala are a major pathway for triggering strong behavioral responses to a variety of odorants. However, while this broad mapping has been established, the patterns of amygdala feedback connectivity and the influence on olfactory circuitry remain unknown. Here, using a combination of neuronal tracing approaches, we dissect the connectivity of a cortical amygdala [posteromedial cortical nucleus (PmCo)] feedback circuit innervating the mouse accessory olfactory bulb. Optogenetic activation of PmCo feedback mainly results in feedforward mitral cell (MC) inhibition through direct excitation of GABAergic granule cells. In addition, LED-driven activity of corticofugal afferents increases ...


Ethical Dilemma Of Alzheimer's Disease And Informed Consent, DaeNia La Rodé 2018 Augustana College, Rock Island Illinois

Ethical Dilemma Of Alzheimer's Disease And Informed Consent, Daenia La Rodé

Augustana Center for the Study of Ethics Essay Contest

No abstract provided.


Motor Subtypes Of Parkinson’S Disease Can Be Identified By Frequency Component Of Postural Stability, Saba Rezvanian, Thurmon Lockhart, Christopher Frames, Rahul Soangra, Abraham Lieberman 2018 Arizona State University

Motor Subtypes Of Parkinson’S Disease Can Be Identified By Frequency Component Of Postural Stability, Saba Rezvanian, Thurmon Lockhart, Christopher Frames, Rahul Soangra, Abraham Lieberman

Physical Therapy Faculty Articles and Research

Parkinson’s disease (PD) can be divided into two subtypes based on clinical features—namely tremor dominant (TD) and postural instability and gait difficulty (PIGD). This categorization is important at the early stage of PD, since identifying the subtypes can help to predict the clinical progression of the disease. Accordingly, correctly diagnosing subtypes is critical in initiating appropriate early interventions and tracking the progression of the disease. However, as the disease progresses, it becomes increasingly difficult to further distinguish those attributes that are relevant to the subtypes. In this study, we investigated whether a method using the standing center of ...


Evaluation Of Group Movement Programs (Plié And Paired Plié) For People With Memory Loss, Alzheimer’S Disease Or Dementia, Martin Blanco, Deborah Barnes 2018 George Washington University

Evaluation Of Group Movement Programs (Plié And Paired Plié) For People With Memory Loss, Alzheimer’S Disease Or Dementia, Martin Blanco, Deborah Barnes

GW Research Days 2016 - Present

Today, one in ten people ages 65 and older have Alzheimer’s dementia. This serious condition often causes great suffering among affected individuals and their families, and there are no medications that stop or slow the disease. It is therefore important to evaluate alternative interventions that may benefit both patients and their care partners. Preventing Loss of Independence through Exercise (PLIÉ) and Paired PLIÉ are group movement programs that combine neuroscience and integrative medicine for individuals with dementia. We analyzed data from [N=39] anonymous evaluation surveys performed as part of two ongoing randomized control trials. Survey data included general ...


Comparison Of Existing Methods For Algorithmic Classification Of Dementia In The Health And Retirement Study, Kan Z. Gianattasio, Qiong Wu, M Maria Glymour, Melinda C. Power 2018 George Washington University

Comparison Of Existing Methods For Algorithmic Classification Of Dementia In The Health And Retirement Study, Kan Z. Gianattasio, Qiong Wu, M Maria Glymour, Melinda C. Power

GW Research Days 2016 - Present

Background: Dementia ascertainment is difficult and costly, hindering the use of large, representative studies such as the Health and Retirement Study (HRS) to monitor trends or disparities in dementia. To address this issue, multiple groups of researchers have developed algorithms to classify dementia status in HRS participants using data from HRS and the Aging, Demographics, and Memory Study (ADAMS), an HRS sub-study that systematically ascertained dementia status. However, the relative performance of each algorithm has not been systematically evaluated.

Objective: To compare the performance of five existing algorithms, overall and by sociodemographic subgroups.

Methods: We created two standardized datasets: (a ...


Vascular Neurocognitive Disorders And The Vascular Risk Factors, Carmen V. Albu, Vlad Padureanu, Mihail V. Boldeanu, Ana M. Bumbea, Anca S. Enescu, Dana M. Albulescu, Cristian A. Silosi, Aurelia Enescu 2018 Craiova University of Medicine and Pharmacy, Department of Neurology

Vascular Neurocognitive Disorders And The Vascular Risk Factors, Carmen V. Albu, Vlad Padureanu, Mihail V. Boldeanu, Ana M. Bumbea, Anca S. Enescu, Dana M. Albulescu, Cristian A. Silosi, Aurelia Enescu

Journal of Mind and Medical Sciences

Dementias are clinical neurodegenerative diseases characterized by permanent and progressive transformation of cognitive functions such as memory, learning capacity, attention, thinking, language, passing judgments, calculation or orientation. Dementias represent a relatively frequent pathology, encountered at about 10% of the population of 65-year olds and 20% of the population of 80-year olds.

This review presents the main etiological forms of dementia, which include Alzheimer form of dementia, vascular dementia, dementia associated with alpha-synucleionopathies, and mixed forms. Regarding vascular dementia, the risk factors are similar to those for an ischemic or hemorrhagic cerebrovascular accident: arterial hypertension, diabetes mellitus, dyslipidemia, smoking, obesity, age ...


Biomarkers Of Rehabilitation Therapy Vary According To Stroke Severity, Erin Burke Quinlan, Lucy Dodakian, Jill See, Alison McKenzie, Jill Campbell Stewart, Steven C. Cramer 2018 University of California, Irvine

Biomarkers Of Rehabilitation Therapy Vary According To Stroke Severity, Erin Burke Quinlan, Lucy Dodakian, Jill See, Alison Mckenzie, Jill Campbell Stewart, Steven C. Cramer

Physical Therapy Faculty Articles and Research

Biomarkers that capture treatment effects could improve the precision of clinical decision making for restorative therapies. We examined the performance of candidate structural, functional,and angiogenesis-related MRI biomarkers before and after a 3-week course of standardized robotic therapy in 18 patients with chronic stroke and hypothesized that results vary significantly according to stroke severity. Patients were 4.1 ± 1 months poststroke, with baseline arm Fugl-Meyer scores of 20–60. When all patients were examined together, no imaging measure changed over time in a manner that correlated with treatment-induced motor gains. However, when also considering the interaction with baseline motor status ...


Cerebral Organoids Derived From Sandhoff Disease-Induced Pluripotent Stem Cells Exhibit Impaired Neurodifferentiation, Maria L. Allende, Emily K. Cook, Bridget C. Larman, Adrienne Nugent, Jacqueline M. Brady, Diane Golebiowski, Miguel Sena-Esteves, Cynthia J. Tifft, Richard L. Proia 2018 National Institutes of Health

Cerebral Organoids Derived From Sandhoff Disease-Induced Pluripotent Stem Cells Exhibit Impaired Neurodifferentiation, Maria L. Allende, Emily K. Cook, Bridget C. Larman, Adrienne Nugent, Jacqueline M. Brady, Diane Golebiowski, Miguel Sena-Esteves, Cynthia J. Tifft, Richard L. Proia

Open Access Articles

Sandhoff disease, one of the GM2 gangliosidoses, is a lysosomal storage disorder characterized by the absence of beta-hexosaminidase A and B activity and the concomitant lysosomal accumulation of its substrate, GM2 ganglioside. It features catastrophic neurodegeneration and death in early childhood. How the lysosomal accumulation of ganglioside might affect the early development of the nervous system is not understood. Recently, cerebral organoids derived from induced pluripotent stem (iPS) cells have illuminated early developmental events altered by disease processes. To develop an early neurodevelopmental model of Sandhoff disease, we first generated iPS cells from the fibroblasts of an infantile Sandhoff disease ...


The Lrrk2 Variant E193k Prevents Mitochondrial Fission Upon Mpp+ Treatment By Altering Lrrk2 Binding To Drp1, Maria Perez Carrion, John E. Landers, Stefano Goldwurm, Giovanni Piccoli 2018 University of Trento

The Lrrk2 Variant E193k Prevents Mitochondrial Fission Upon Mpp+ Treatment By Altering Lrrk2 Binding To Drp1, Maria Perez Carrion, John E. Landers, Stefano Goldwurm, Giovanni Piccoli

Open Access Articles

Mutations in leucine-rich repeat kinase 2 gene (LRRK2) are associated with familial and sporadic Parkinson's disease (PD). LRRK2 is a complex protein that consists of multiple domains, including 13 putative armadillo-type repeats at the N-terminus. In this study, we analyzed the functional and molecular consequences of a novel variant, E193K, identified in an Italian family. E193K substitution does not influence LRRK2 kinase activity. Instead it affects LRRK2 biochemical properties, such as phosphorylation at Ser935 and affinity for 14-3-3epsilon. Primary fibroblasts obtained from an E193K carrier demonstrated increased cellular toxicity and abnormal mitochondrial fission upon 1-methyl-4-phenylpyridinium treatment. We found that ...


Identification Of Novel Genetic Variations For Amyotrophic Lateral Sclerosis (Als), Guang Xu 2018 University of Massachusetts Medical School

Identification Of Novel Genetic Variations For Amyotrophic Lateral Sclerosis (Als), Guang Xu

GSBS Dissertations and Theses

A list of genes have been identified to carry mutations causing familial ALS such as SOD1, TARDBP, C9orf72. But for sporadic ALS, which is 90% of all ALS cases, the underlying genetic variants are still largely unknown. There are multiple genome-wide association study (GWAS) for sporadic ALS, but usually a large number nominated SNP can hardly be replicated in larger cohort analysis. Also majority of GWAS SNP lie within noncoding region of genome, imposing a huge challenge to study their biological role in ALS pathology. With the rapid development of next-generation sequencing technology, we are able to sequence exome and ...


A Rare Case Of Enhancing Anterior Interhemispheric Racemose Neurocysticercosis With Foci Of Calcification And Direct Parenchymal Extension, Anupama Chandrappa, Rajesh Raman, Suchetha Satish 2018 JSS Medical College Hospital, JSS University, Mysore

A Rare Case Of Enhancing Anterior Interhemispheric Racemose Neurocysticercosis With Foci Of Calcification And Direct Parenchymal Extension, Anupama Chandrappa, Rajesh Raman, Suchetha Satish

Journal of Global Radiology

Neurocysticercosis (NCC) refers to the central nervous system (CNS) infestation of the larvae of pork tapeworm Taenia solium. It is endemic in Central and South America, Asia and Africa. It most commonly presents as intraparenchymal subcentimetric ring enhancing lesions of variable ages (or size and duration) with surrounding vasogenic edema. The lesions calcify with time. Rarely, NCC can occur in extraparenchymal locations, within the subarachnoid space and ventricles. At these locations it is usually seen as a proliferative lobulated cystic mass without a scolex which is termed racemose neurocysticercosis. Racemose cysticerci do not show calcification, scolex, or enhancement.

We report ...


A Cre-Inducible Dux4 Transgenic Mouse Model For Investigating Facioscapulohumeral Muscular Dystrophy, Takako I. Jones, Peter L. Jones 2018 University of Massachusetts Medical School

A Cre-Inducible Dux4 Transgenic Mouse Model For Investigating Facioscapulohumeral Muscular Dystrophy, Takako I. Jones, Peter L. Jones

Open Access Articles

The Double homeobox 4 (DUX4) gene is an important regulator of early human development and its aberrant expression is causal for facioscapulohumeral muscular dystrophy (FSHD). The DUX4-full length (DUX4-fl) mRNA splice isoform encodes a transcriptional activator; however, DUX4 and its unique DNA binding preferences are specific to old-world primates. Regardless, the somatic cytotoxicity caused by DUX4 expression is conserved when expressed in cells and animals ranging from fly to mouse. Thus, viable animal models based on DUX4-fl expression have been difficult to generate due in large part to overt developmental toxicity of low DUX4-fl expression from leaky transgenes. We have ...


(18)F-Vc701-Pet And Mri In The In Vivo Neuroinflammation Assessment Of A Mouse Model Of Multiple Sclerosis, Sara Belloli, Lucia Zanotti, Valentina Murtaj, Cristina Mazzon, Giuseppe Di Grigoli, Cristina Monterisi, Valeria Masiello, Leonardo Iaccarino, Andrea Cappelli, Pietro Luigi Poliani, Letterio S. Politi, Rosa Maria Moresco 2018 University of Milano-Bicocca

(18)F-Vc701-Pet And Mri In The In Vivo Neuroinflammation Assessment Of A Mouse Model Of Multiple Sclerosis, Sara Belloli, Lucia Zanotti, Valentina Murtaj, Cristina Mazzon, Giuseppe Di Grigoli, Cristina Monterisi, Valeria Masiello, Leonardo Iaccarino, Andrea Cappelli, Pietro Luigi Poliani, Letterio S. Politi, Rosa Maria Moresco

Radiology Publications and Presentations

BACKGROUND: Positron emission tomography (PET) using translocator protein (TSPO) ligands has been used to detect neuroinflammatory processes in neurological disorders, including multiple sclerosis (MS). The aim of this study was to evaluate neuroinflammation in a mouse MS model (EAE) using TSPO-PET with (18)F-VC701, in combination with magnetic resonance imaging (MRI).

METHODS: MOG35-55/CFA and pertussis toxin protocol was used to induce EAE in C57BL/6 mice. Disease progression was monitored daily, whereas MRI evaluation was performed at 1, 2, and 4 weeks post-induction. Microglia activation was assessed in vivo by (18)F-VC701 PET at the time of maximum disease ...


Pilot Study To Assess Visualization And Therapy Of Inflammatory Mechanisms After Vessel Reopening In A Mouse Stroke Model, Ebba Beller, Laura Reuter, Anne Kluge, Christine Preibisch, Ute Lindauer, Alexei A. Bogdanov Jr., Friederike Lammer, Claire Delbridge, Kaspar Matiasek, Benedikt J. Schwaiger, Tobias Boeckh-Behrens, Claus Zimmer, Alexandra S. Gersing 2018 Klinikum rechts der Isar der Technischen Universität München

Pilot Study To Assess Visualization And Therapy Of Inflammatory Mechanisms After Vessel Reopening In A Mouse Stroke Model, Ebba Beller, Laura Reuter, Anne Kluge, Christine Preibisch, Ute Lindauer, Alexei A. Bogdanov Jr., Friederike Lammer, Claire Delbridge, Kaspar Matiasek, Benedikt J. Schwaiger, Tobias Boeckh-Behrens, Claus Zimmer, Alexandra S. Gersing

Radiology Publications and Presentations

After reperfusion therapy in stroke patients secondary inflammatory processes may increase cerebral damage. In this pilot study, effects of anti-inflammatory therapy were assessed in a middle cerebral artery occlusion (MCAO) mouse model after reperfusion. 1 hour after MCAO, the artery was reopened and tacrolimus or NaCl were administered intra-arterially. Perfusion-weighted (PWI) and diffusion-weighted images (DWI) were obtained by MRI during MCAO. DWI, T2- and T1-weighted images with and without Bis-5HT-DTPA administration were obtained 24 hours after MCAO. Neutrophils, Myeloperoxidase-positive-(MPO+)-cells and microglia, including M1 and M2 phenotypes, were assessed immunohistochemically. Treatment with tacrolimus led to significantly smaller apparent diffusion ...


C9orf72 Ggggcc Repeat-Associated Non-Aug Translation Is Upregulated By Stress Through Eif2alpha Phosphorylation, Weiwei Cheng, Shaopeng Wang, Alexander A. Mestre, Chenglai Fu, Andres Makarem, Fengfan Xian, Lindsey R. Hayes, Rodrigo Lopez-Gonzalez, Kevin Drenner, Jie Jiang, Don W. Cleveland, Shuying Sun 2018 Johns Hopkins University

C9orf72 Ggggcc Repeat-Associated Non-Aug Translation Is Upregulated By Stress Through Eif2alpha Phosphorylation, Weiwei Cheng, Shaopeng Wang, Alexander A. Mestre, Chenglai Fu, Andres Makarem, Fengfan Xian, Lindsey R. Hayes, Rodrigo Lopez-Gonzalez, Kevin Drenner, Jie Jiang, Don W. Cleveland, Shuying Sun

Open Access Articles

Hexanucleotide repeat expansion in C9ORF72 is the most frequent cause of both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Here we demonstrate that the repeat-associated non-AUG (RAN) translation of (GGGGCC) n -containing RNAs into poly-dipeptides can initiate in vivo without a 5'-cap. The primary RNA substrate for RAN translation of C9ORF72 sense repeats is shown to be the spliced first intron, following its excision from the initial pre-mRNA and transport to the cytoplasm. Cap-independent RAN translation is shown to be upregulated by various stress stimuli through phosphorylation of the alpha subunit of eukaryotic initiation factor-2 (eIF2alpha), the core ...


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