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Mitochondrial Aspects Of Neuronal Pathology In Triple-Transgenic Alzheimer’S Disease Mice, John Zachary Cavendish 2021 West Virginia University

Mitochondrial Aspects Of Neuronal Pathology In Triple-Transgenic Alzheimer’S Disease Mice, John Zachary Cavendish

Graduate Theses, Dissertations, and Problem Reports

Alzheimer’s disease (AD) is a fatal, progressive neurodegenerative disease afflicting millions of people in the United States alone and is the only one of the top leading causes of morbidity and mortality with no effective disease-modifying therapies. It is the most common form of dementia, affecting one in three people over the age of 85. While the hallmarks of the disease include accumulation of beta-amyloid-based extracellular plaques and hyperphosphorylated tau-based intracellular neurofibrillary tangles, treatment strategies centered on removing or mitigating these components of AD have all failed in humans. Mitochondrial dysfunction has been increasingly recognized as an early and ...


Functions Of Fibroblast Growth Factor Homologous Factor 2 In Excitable Tissues, Christopher Marra 2020 The Graduate Center, City University of New York

Functions Of Fibroblast Growth Factor Homologous Factor 2 In Excitable Tissues, Christopher Marra

Dissertations, Theses, and Capstone Projects

Purpose: Fibroblast Growth Factor Homologous Factors (FHFs) are a group of proteins known to associate with and modulate voltage-gated sodium channels (Nav) in excitable cells. The four FHF genes are differentially expressed in specific cell-types, with FHF2 expressed prominently in the hippocampus, cerebral cortex, heart and dorsal root ganglia. Due to previous unavailability of an Fhf2 knockout mouse, this gene’s functions have been understudied in comparison to other those encoding other FHFs. The purpose of this research has been to better understand the normal physiological functions of FHF2 at the cellular and system levels in the heart, sensory ...


Methylmercury Cytotoxicity On Developing Neuronal Lineages And Differences In Susceptibility Based On Media Type, Madeline Henley 2020 Purdue University

Methylmercury Cytotoxicity On Developing Neuronal Lineages And Differences In Susceptibility Based On Media Type, Madeline Henley

The Journal of Purdue Undergraduate Research

No abstract provided.


Characterization Of Neuronal Differentiation And Activity In Human-Induced Pluripotent Neural Stem Cells, Allison Biddinger 2020 Purdue University

Characterization Of Neuronal Differentiation And Activity In Human-Induced Pluripotent Neural Stem Cells, Allison Biddinger

The Journal of Purdue Undergraduate Research

No abstract provided.


The Role Of The Leucine-Rich (Leur) Domain Of Rho Guanine Nucleotide Exchange Factor (Rgnef) In The Regulation Of Amyotrophic Lateral Sclerosis (Als) Associated Protein Tar Dna-Binding Protein Of 43 Kda (Tdp-43), Hind Amzil 2020 The University of Western Ontario

The Role Of The Leucine-Rich (Leur) Domain Of Rho Guanine Nucleotide Exchange Factor (Rgnef) In The Regulation Of Amyotrophic Lateral Sclerosis (Als) Associated Protein Tar Dna-Binding Protein Of 43 Kda (Tdp-43), Hind Amzil

Electronic Thesis and Dissertation Repository

The presence of neuronal cytoplasmic inclusions (NCIs) composed of RNA-binding proteins (RBPs) and neurofilaments is considered to be ALS’s neuropathological hallmark. RGNEF has been previously shown to interact with TDP-43 and to have a regulatory effect on the expression levels of NEFL mRNA and NFL protein in vitro. Here, I examined the mechanism of the RGNEF N-terminus, leucine-rich domain (LeuR) domain’s interaction with TDP-43. I observed that the minimal domain required is 110 amino acids (LeuR110), that the Ankyrin domain adjacent to LeuR110 does not participate, and that LeuR110 forms of a high molecular weight complex with TDP-43 ...


Methods For Detecting Per2::Luciferase Bioluminescence Rhythms In Freely Moving Mice, B. Martin-Burgos, W. Wang, I. William, S. Tir, I. Mohammad, R. Javed, Smith College, Y. Cui, Ciearra B. Smith, V. van der Vinne, P.C. Molyneux, Stephen C. Miller, David R. Weaver, T.L. Leise, M.E. Harrington 2020 Smith College

Methods For Detecting Per2::Luciferase Bioluminescence Rhythms In Freely Moving Mice, B. Martin-Burgos, W. Wang, I. William, S. Tir, I. Mohammad, R. Javed, Smith College, Y. Cui, Ciearra B. Smith, V. Van Der Vinne, P.C. Molyneux, Stephen C. Miller, David R. Weaver, T.L. Leise, M.E. Harrington

University of Massachusetts Medical School Faculty Publications

Circadian rhythms are driven by daily oscillations of gene expression. An important tool for studying cellular and tissue rhythms is the use of a gene reporter, such as bioluminescence from the reporter gene luciferase controlled by a rhythmically expressed gene of interest. Here we describe methods that allow measurement of bioluminescence from a freely-moving mouse housed in a standard cage. Using a LumiCycle In Vivo (Actimetrics), we determined conditions that allow detection of circadian rhythms of bioluminescence from the PER2 reporter, PER2::LUC, in freely behaving mice. We tested delivery of D-luciferin via a subcutaneous minipump and in the drinking ...


Altered Micos Morphology And Mitochondrial Ion Homeostasis Contribute To Poly(Gr) Toxicity Associated With C9-Als/Ftd, Shuangxi Li, Zhihao Wu, Yu Li, Ishaq Tantray, Diego De Stefani, Andrea Mattarei, Gopinath Krishnan, Fen-Biao Gao, Hannes Vogel, Bingwei Lu 2020 Stanford University

Altered Micos Morphology And Mitochondrial Ion Homeostasis Contribute To Poly(Gr) Toxicity Associated With C9-Als/Ftd, Shuangxi Li, Zhihao Wu, Yu Li, Ishaq Tantray, Diego De Stefani, Andrea Mattarei, Gopinath Krishnan, Fen-Biao Gao, Hannes Vogel, Bingwei Lu

Open Access Articles

Amyotrophic lateral sclerosis (ALS) manifests pathological changes in motor neurons and various other cell types. Compared to motor neurons, the contribution of the other cell types to the ALS phenotypes is understudied. G4C2 repeat expansion in C9ORF72 is the most common genetic cause of ALS along with frontotemporal dementia (C9-ALS/FTD), with increasing evidence supporting repeat-encoded poly(GR) in disease pathogenesis. Here, we show in Drosophila muscle that poly(GR) enters mitochondria and interacts with components of the Mitochondrial Contact Site and Cristae Organizing System (MICOS), altering MICOS dynamics and intra-subunit interactions. This impairs mitochondrial inner membrane structure, ion homeostasis ...


Rela/P65 Blocks Histone Deacetylase-3 Neurotoxicity And Protects Neurons Against Neuronal Death Induced By Polyq-Expanded Huntingtin And Ataxin-1 In A P65 Phospho S276 Dependent Manner, Yiyu Zhang 2020 Southern Methodist University

Rela/P65 Blocks Histone Deacetylase-3 Neurotoxicity And Protects Neurons Against Neuronal Death Induced By Polyq-Expanded Huntingtin And Ataxin-1 In A P65 Phospho S276 Dependent Manner, Yiyu Zhang

Biological Sciences Theses and Dissertations

Neurodegenerative diseases have a large negative impact to human society. Symptoms of neurodegenerative diseases includes memory loss, impaired recognition, motor dysfunction due to dysregulated neuronal loss in different brain regions. However, the neurobiological basis of these brain diseases is not fully understood and there are no cures or effective treatments. Polyglutamine (Poly-Q) disorders is a class of neurodegenerative diseases that are caused by polyglutamine expansion within the protein coding regions of specific genes. Huntington’s disease (HD), Spinal Cerebellar Ataxia Type 1 (SCA1) and Spinal Cerebellar Ataxia Type 3 (SCA3) are three common diseases among Poly-Q disorders. To better understand ...


The Role Of Skeletal Muscle-Synthesized Brain Derived Neurotrophic Factor In The Maintenance Of Motor Neuron Mitochondrial Populations, Mikel Cawley 2020 Northern Michigan University

The Role Of Skeletal Muscle-Synthesized Brain Derived Neurotrophic Factor In The Maintenance Of Motor Neuron Mitochondrial Populations, Mikel Cawley

All NMU Master's Theses

Mitochondria are essential for the high energy demands of the neuromuscular junction and, as a consequence, leave motorneurons susceptible to dysfunction. A potential origin of progressive pathology may be a reduction in brain-derived neurotrophic-factor (BDNF) signaling at the motor unit. We have shown that mice deficient in skeletal muscle-synthesized BDNF (msBDNF) demonstrate progressive motorneuron and muscle pathology at 120d. We hypothesize mitochondrial populations will be altered in motorneurons of msBDNF deficient-mice. At 117d, msBDNF deficient-mice received intramuscular injections of MitoTracker™ dye targeting the right gastrocnemius muscle. At 120d experimental groups underwent a gastrocnemius harvest or a sciatic nerve ligation protocol ...


Loss Of Supervillin Causes Myopathy With Myofibrillar Disorganization And Autophagic Vacuoles, Carola Hedberg-Oldfors, Elizabeth J. Luna, Anders Oldfors, Cordula Knopp 2020 University of Gothenburg

Loss Of Supervillin Causes Myopathy With Myofibrillar Disorganization And Autophagic Vacuoles, Carola Hedberg-Oldfors, Elizabeth J. Luna, Anders Oldfors, Cordula Knopp

Open Access Articles

The muscle specific isoform of the supervillin protein (SV2), encoded by the SVIL gene, is a large sarcolemmal myosin II- and F-actin-binding protein. Supervillin (SV2) binds and co-localizes with costameric dystrophin and binds nebulin, potentially attaching the sarcolemma to myofibrillar Z-lines. Despite its important role in muscle cell physiology suggested by various in vitro studies, there are so far no reports of any human disease caused by SVIL mutations. We here report four patients from two unrelated, consanguineous families with a childhood/adolescence onset of a myopathy associated with homozygous loss-of-function mutations in SVIL. Wide neck, anteverted shoulders and prominent ...


Regulation Of Translation And Synaptic Plasticity By Tsc2, Annie Hien 2020 University of Massachusetts Medical School

Regulation Of Translation And Synaptic Plasticity By Tsc2, Annie Hien

GSBS Dissertations and Theses

Mutations in TSC2 cause the disorder tuberous sclerosis (TSC), which has a high incidence of autism and intellectual disability. TSC2 regulates mRNA translation required for group 1 metabotropic glutamate receptor-dependent synaptic long-term depression (mGluR-LTD), but the identity of mRNAs responsive to mGluR-LTD signaling in the normal and TSC brain is largely unknown. We generated Tsc2+/- mice to model TSC autism and performed ribosome profiling to identify differentially expressed genes following mGluR-LTD in the normal and Tsc2+/- hippocampus. Ribosome profiling reveals that in Tsc2+/-mice, RNA-binding targets of Fragile X Mental Retardation Protein (FMRP) are increased. In wild-type hippocampus, induction of ...


Ampa Receptor Auxiliary Subunit Gsg1l Suppresses Short-Term Facilitation In Corticothalamic Synapses And Determines Seizure Susceptibility, Aichurok Kamalova, Kensuke Futai, Eric Delpire, Terunaga Nakagawa 2020 Vanderbilt University

Ampa Receptor Auxiliary Subunit Gsg1l Suppresses Short-Term Facilitation In Corticothalamic Synapses And Determines Seizure Susceptibility, Aichurok Kamalova, Kensuke Futai, Eric Delpire, Terunaga Nakagawa

Open Access Articles

The anterior thalamus (AT) is critical for memory formation, processing navigational information, and seizure initiation. However, the molecular mechanisms that regulate synaptic function of AT neurons remain largely unexplored. We report that AMPA receptor auxiliary subunit GSG1L controls short-term plasticity in AT synapses that receive inputs from the cortex, but not in those receiving inputs from other pathways. A canonical auxiliary subunit stargazin co-exists in these neurons but is functionally absent from corticothalamic synapses. In GSG1L knockout mice, AT neurons exhibit hyperexcitability and the animals have increased susceptibility to seizures, consistent with a negative regulatory role of GSG1L. We hypothesize ...


Kcnq2 Localization In The Brainstem, Christina Valera 2020 University of Connecticut

Kcnq2 Localization In The Brainstem, Christina Valera

Honors Scholar Theses

KCNQ2 channels are potassium channels that serve to control neuronal excitability. Loss of function mutations in these channels are known to cause various forms of epilepsy. Recently, KCNQ2 R201C and R201H gain of function mutations have been shown to exhibit an exaggerated startle response and other unique phenotypes uncharacteristic of epilepsy. These phenotypes resemble hyperekplexia, a condition in which glycine neurotransmission in the spinal cord and brainstem is affected. While KCNQ2 has widespread localization throughout the brain, its presence in the brainstem remains unknown. We used immunostaining to determine the localization of KCNQ2 in the vagus nerve and hypoglossal nerve ...


Association Of Dtnbp1 With Schizophrenia: Findings From Two Independent Samples Of Han Chinese Population, Yongfeng Yang, Xiaoduo Fan, Ge Yang 2020 Xinxiang Medical University

Association Of Dtnbp1 With Schizophrenia: Findings From Two Independent Samples Of Han Chinese Population, Yongfeng Yang, Xiaoduo Fan, Ge Yang

Psychiatry Publications

Objectives: Schizophrenia (SZ) is a complex psychiatric disorder that has a strong genetic basis. Dystrobrevin-binding protein 1 (DTNBP1) is one of the genes thought to be pivotal in regulating the glutamatergic system. Studies have suggested that variations in DTNBP1 confer susceptibility to SZ and clinical symptoms. Here, we performed a two-stage independent verification study to identify polymorphisms of the DTNBP1 gene that might be associated with SZ in the Han Chinese population.

Methods: In stage 1, 14 single nucleotide polymorphisms (SNPs) were genotyped in 528 paranoid SZ patients and 528 healthy controls (HCs) using the Illumina GoldenGate assays on a ...


Yap1/Taz Drives Ependymoma-Like Tumour Formation In Mice, Noreen Eder, Federico Roncaroli, Marie-Charlotte Dolmart, Stuart Horswell, Felipe Andreiuolo, Helen R. Flynn, Andre T. Lopes, Suzanne Claxton, John-Paul Kilday, Lucy Collinson, Junhao Mao, Torsten Pietsch, Barry Thompson, Ambrosius P. Snijders, Sila K. Ultanir 2020 The Francis Crick Institute

Yap1/Taz Drives Ependymoma-Like Tumour Formation In Mice, Noreen Eder, Federico Roncaroli, Marie-Charlotte Dolmart, Stuart Horswell, Felipe Andreiuolo, Helen R. Flynn, Andre T. Lopes, Suzanne Claxton, John-Paul Kilday, Lucy Collinson, Junhao Mao, Torsten Pietsch, Barry Thompson, Ambrosius P. Snijders, Sila K. Ultanir

Open Access Articles

YAP1 gene fusions have been observed in a subset of paediatric ependymomas. Here we show that, ectopic expression of active nuclear YAP1 (nlsYAP5SA) in ventricular zone neural progenitor cells using conditionally-induced NEX/NeuroD6-Cre is sufficient to drive brain tumour formation in mice. Neuronal differentiation is inhibited in the hippocampus. Deletion of YAP1's negative regulators LATS1 and LATS2 kinases in NEX-Cre lineage in double conditional knockout mice also generates similar tumours, which are rescued by deletion of YAP1 and its paralog TAZ. YAP1/TAZ-induced mouse tumours display molecular and ultrastructural characteristics of human ependymoma. RNA sequencing and quantitative proteomics of ...


Expression Analyses Of Hippocampal And Cortical Proteins In A Rat Model For Alzheimer’S Disease, Rangon Islam 2020 CUNY Hunter College

Expression Analyses Of Hippocampal And Cortical Proteins In A Rat Model For Alzheimer’S Disease, Rangon Islam

School of Arts & Sciences Theses

Currently, Alzheimer’s disease (AD) has no cure. Using a rat AD model, we identified aberrantly expressed proteins during pre-pathology as potential biomarkers. The expression of certain biomarkers was reversed by diazoxide, a repurposed hypertension drug. These results suggest that drug repurposing at an early stage of AD has therapeutic potential.


Hiv-1 Tat Interactions With Opioids Are Modulated By Progesterone And Estradiol, Dejun Jackson 2020 University of Mississippi

Hiv-1 Tat Interactions With Opioids Are Modulated By Progesterone And Estradiol, Dejun Jackson

Honors Theses

HIV infection and combined substance abuse are comorbid epidemics. Previous studies show that concurrent opioid drug use may potentiate HIV-1-mediated neurotoxicity partly via interactions with opioids. Preclinical studies suggest that the HIV-1 trans-activator of transcription (Tat), an HIV regulatory protein, can synergize with opioids to exacerbate its already neurotoxic effects. However, its interactions with clinical opioids, such as oxycodone, have yet to be elucidated. Additionally, Tat disrupts a number of systems including the dopaminergic system, which contribute to its capacity to potentiate the rewarding effects of abused drugs. Although the neurotoxic effects of Tat may be inhibited by gonadal steroids ...


Neuroligin3 Splice Isoforms Shape Inhibitory Synaptic Function In The Mouse Hippocampus, Motokazu Uchigashima, Ming Leung, Takuya Watanabe, Amy Cheung, Timmy Le, Sabine Pallat, Alexandre Luis Marques Dinis, Masahiko Watanabe, Yuka Imamura Kawasawa, Kensuke Futai 2020 University of Massachusetts Medical School

Neuroligin3 Splice Isoforms Shape Inhibitory Synaptic Function In The Mouse Hippocampus, Motokazu Uchigashima, Ming Leung, Takuya Watanabe, Amy Cheung, Timmy Le, Sabine Pallat, Alexandre Luis Marques Dinis, Masahiko Watanabe, Yuka Imamura Kawasawa, Kensuke Futai

Open Access Articles

Synapse formation is a dynamic process essential for the development and maturation of the neuronal circuitry in the brain. At the synaptic cleft, transsynaptic protein-protein interactions are major biological determinants of proper synapse efficacy. The balance of excitatory and inhibitory synaptic transmission (E-I balance) stabilizes synaptic activity, and dysregulation of the E-I balance has been implicated in neurodevelopmental disorders, including autism spectrum disorders. However, the molecular mechanisms underlying the E-I balance remain to be elucidated. Here, using single-cell transcriptomics, immunohistochemistry and electrophysiology approaches to murine CA1 pyramidal neurons obtained from organotypic hippocampal slice cultures, we investigate Neuroligin (Nlgn) genes that ...


The Current Neuroscientific Understanding Of Alzheimer's Disease, Rachel A. Brandes 2020 University of Tennessee, Knoxville

The Current Neuroscientific Understanding Of Alzheimer's Disease, Rachel A. Brandes

Pursuit - The Journal of Undergraduate Research at the University of Tennessee

Alzheimer’s disease is a degenerative neurological illness characterized by the deterioration of brain regions implicated in memory and cognitive function. While researchers have yet to find a cure or effective treatment, they have gained a better understanding of its pathology and development. Through years of neuroscience research, scientists have discovered much of what happens in the brain during Alzheimer’s disease onset and how this causes its symptoms; many hypotheses regarding this aspect of the illness involve temporal lobe atrophy, neurofibrillary tangles, and amyloid plaques. Although Alzheimer’s disease affects millions of people every day, it seems that most ...


Neurocognitive Risk Factors And Current Intervention Strategies For Survivors Of Pediatric Acute Lymphoblastic Leukemia, Abigail Taber 2020 Liberty University

Neurocognitive Risk Factors And Current Intervention Strategies For Survivors Of Pediatric Acute Lymphoblastic Leukemia, Abigail Taber

Senior Honors Theses

The improved survival rate for pediatric cancer patients is one of the greatest triumphs of recent medicine, but the late effects faced by these survivors have been uncovered through this new population of survivors. Many survivors of pediatric acute lymphoblastic leukemia (ALL) experience cognitive deficits in areas such as attention, memory, processing speed, and academic achievement following cancer treatment. Recent research has pointed to chemotherapeutic agents, host risk factors, and genetic predispositions as perpetrators of these deficits, although other factors are also under investigation. Consequently, the search for appropriate interventions for the amelioration of these deficits has dominated the literature ...


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