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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 [Preprint], 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 [Preprint], 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 Publications by UMMS Authors

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 ...


Cellular And Circuit Properties Of Slow Oscillations In The Thalamic Reticular Nucleus, John O'Malley 2020 The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences

Cellular And Circuit Properties Of Slow Oscillations In The Thalamic Reticular Nucleus, John O'Malley

The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences Dissertations and Theses (Open Access)

During sleep, neurons in the thalamic reticular nucleus (TRN) generate distinct types of oscillatory activity. While the reciprocal synaptic circuits between TRN and sensory thalamic nuclei underlie the generation of sleep spindles, the mechanisms regulating slow (<1 >Hz) forms of thalamic oscillations are poorly understood. Under in vitro conditions, in the absence of synaptic inputs, TRN neurons can generate slow oscillations in a cell-intrinsic manner. Activation of postsynaptic Group 1 metabotropic glutamate receptors (mGluR) leads to long-lasting plateau potentials thought to be mediated by both T-type calcium currents and calcium-activated nonselective cation currents (ICAN). However, the identity of ICAN ...


Elevated Cochlear Adenosine Causes Hearing Loss Via Adora2b Signaling, Jeanne Manalo 2020 The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences

Elevated Cochlear Adenosine Causes Hearing Loss Via Adora2b Signaling, Jeanne Manalo

The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences Dissertations and Theses (Open Access)

Over 538 million people in the world have been diagnosed with hearing loss (HL). Current treatments for the most common type of HL, sensorineural HL, are limited to hearing aids and cochlear implants with no FDA-drugs available. The hearing process demands an abundance of ATP and HL is often attributed to a disruption in this metabolic energy currency. Patients who lack adenosine deaminase (ADA), the enzyme that irreversibly metabolizes adenosine, have high levels of adenosine that yield severe health problems, including HL; however, the pathogenic mechanisms behind HL and adenosine remain elusive. Our lab has found a HL phenotype in ...


Aging Lowers Pex5 Levels In Cortical Neurons In Male And Female Mouse Brains, Ndidi-Ese Uzor 2020 The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences

Aging Lowers Pex5 Levels In Cortical Neurons In Male And Female Mouse Brains, Ndidi-Ese Uzor

The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences Dissertations and Theses (Open Access)

Peroxisomes are small organelles with critical functions: lipid synthesis, breakdown of reactive oxygen species by antioxidant enzymes, and amino acid degradation. In the brain, peroxisomal lipids make up the myelin sheath. Brain peroxisomal dysfunction leads to lipid disruption or neurological consequences if key peroxisomal proteins are absent. Still, it is unclear how peroxisomes are affected in neurodegenerative diseases and in normal brain aging. This work examines peroxisomal markers in three settings: 1) in a neuronal and 2) animal model of Huntington disease (HD), where mutant huntingtin (mHtt), the causative protein in Huntington disease pathogenesis is expressed, and 3) in the ...


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 Publications by UMMS Authors

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 ...


Astrocytes Rescue Neuronal Health After Cisplatin Treatment Through Mitochondrial Transfer., Krystal English, Krystal English 2020 The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences

Astrocytes Rescue Neuronal Health After Cisplatin Treatment Through Mitochondrial Transfer., Krystal English, Krystal English

The University of Texas MD Anderson Cancer Center UTHealth Graduate School of Biomedical Sciences Dissertations and Theses (Open Access)

Abstract

Astrocytes rescue neuronal health after cisplatin treatment through mitochondrial transfer.

Author: Krystal English

Advisory Professor: Dr. Cobi J. Heijnen, Ph.D.

Chemotherapy-induced cognitive impairments are associated with neuronal mitochondrial dysfunction. Cisplatin, a commonly used chemotherapeutic, induces neuronal mitochondrial dysfunction in vivo and in vitro. Astrocytes are key players in supporting neuronal development, synaptogenesis, axonal growth, metabolism and, potentially, mitochondrial health. We tested the hypothesis that astrocytes transfer healthy mitochondria to neurons after cisplatin treatment to restore neuronal health.

We used an in vitro system in which astrocytes with Mito-mCherry-labeled mitochondria were co-cultured with primary cortical neurons or neuronal stem ...


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 Publications by UMMS Authors

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 ...


How Do Adult Songbirds Learn New Sounds? Using Neuromodulators To Probe The Function Of The Auditory Association Cortex, Matheus Macedo-Lima 2020 University of Massachusetts Amherst

How Do Adult Songbirds Learn New Sounds? Using Neuromodulators To Probe The Function Of The Auditory Association Cortex, Matheus Macedo-Lima

Doctoral Dissertations

The ability to associate sounds and outcomes is vital in the life history of many species. Animals constantly assess the soundscape for cues associated with threats, competitors, allies, mates or prey, and experience is crucial for those associations. For vocal learning species such as humans and songbirds, learning sounds (i.e. perception and association learning) is also the first step in the process of vocal learning. Auditory learning is thought to depend on high-order cortical brain structures, where sounds and meaning are bound. In songbirds, the caudomedial nidopallium (NCM) is part of the auditory association cortex and is known to ...


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 Publications by UMMS Authors

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.


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