Open Access. Powered by Scholars. Published by Universities.®
Molecular and Cellular Neuroscience Commons™
Open Access. Powered by Scholars. Published by Universities.®
- Institution
- Keyword
-
- 5-HT4 (1)
- Amyotrophic Lateral Sclerosis (1)
- Anti-amyloid (1)
- Anti-prion (1)
- Antidepressants (1)
-
- Anxiety (1)
- CK2 (1)
- Cholesterol (1)
- Depression (1)
- Desmosterol (1)
- Dhcr24 (1)
- Drosophila (1)
- Drosophila melanogaster (1)
- EBP (1)
- Epigenetics (1)
- Epilepsy (1)
- Fused in Sarcoma (1)
- Gene Activation (1)
- Gene Editing (1)
- GlyRα1, GlyRα2, GlyRα3, GlyRα4 (1)
- Glycine receptor kinetics (1)
- Glycogen (1)
- Human Cells Disease Models (1)
- Huntington's Disease (1)
- Inhibitory retinal circuits (1)
- Lafora disease (1)
- Lung Cancer (1)
- Memory (1)
- Neuroblast (1)
- Neurodegenerative disease (1)
Articles 1 - 9 of 9
Full-Text Articles in Molecular and Cellular Neuroscience
Cholesterol Biosynthesis In The Nervous System With An Emphasis On Desmosterolosis, Luke Allen
Cholesterol Biosynthesis In The Nervous System With An Emphasis On Desmosterolosis, Luke Allen
Theses & Dissertations
Cholesterol biosynthesis is integral to proper neurodevelopment due to the reliance on de novo synthesis of cholesterol in the brain. Disruptions in this process have devastating outcomes for human life characterized by several phenotypic manifestations concomitant with developmental delay. The cholesterol biosynthesis disorder desmosterolosis is an extremely rare disorder with a severe clinical phenotype, however, the models used to study this disease are not well characterized. In addition to genetic disruptions in cholesterol biosynthesis, pharmacological perturbation is an understudied side effect of many commonly prescribed drugs. Here we present a characterization of the sterol profile of the mouse model of …
Targeted Genome-Scale Gene Activation And Gene Editing In Human Cells To Understand Disease Models, Michael De La Cruz
Targeted Genome-Scale Gene Activation And Gene Editing In Human Cells To Understand Disease Models, Michael De La Cruz
KGI Theses and Dissertations
Since the discovery of sequence directed DNA editing reagents such as CRISPR-Cas9 RNA-guided and TALEN DNA endonucleases, there has been a snowball of advances in the life sciences due to the ability to efficiently edit and control genomes within living cells. CRISPR-Cas9 based genomic tools, which facilitate the high-throughput precise manipulation of genes, allow for unbiased functional genomic screens. We used a human CRISPR-Cas9 Synergistic Activation Mediator pooled library which utilizes an engineered protein complex for transcriptional activation of 23,430 endogenous genes to investigate the development of novel resistance mechanisms to lung cancer targeted therapy, Erlotinib. We set out to …
Development Of A High-Throughput System For Screening Of Anti-Prion Molecules, Katherine Do
Development Of A High-Throughput System For Screening Of Anti-Prion Molecules, Katherine Do
Dissertations & Theses (Open Access)
The misfolded prion protein causes and transmits disease in both humans and animals. As other infectious agents, prions display strain variation, which can generate different pathological outcomes in affected individuals. Unfortunately, there are no known therapies for these diseases, which at present are invariably fatal. In this work, the Protein Misfolding Cyclic Amplification technology (PMCA, an in vitro test that replicates minimum quantities of infectious prions) has been modified to screen for small molecules inhibiting prion protein misfolding in a strain-specific manner. In order to approach a high-throughput PMCA system, technical aspects in PMCA has been optimized for application of …
Glycine Receptor Expression Across Identified Retinal Ganglion Cell Types., Ian Scot Pyle
Glycine Receptor Expression Across Identified Retinal Ganglion Cell Types., Ian Scot Pyle
Electronic Theses and Dissertations
Retinal ganglion cells (RGCs) represent the culmination of all retinal signaling and their output forms the substrate for vision throughout the rest of the brain. About 40 different RGC types have been defined by differences in their visually evoked responses, morphology, and genetic makeup. These responses arise from interactions between inhibition and excitation throughout the retinal circuit (Franke et al., 2017; Masland, 2012; Sanes & Masland, 2015; Werblin, 2011). Unlike most other areas of the central nervous system (CNS), the retina utilizes both GABA and glycine inhibitory neurotransmitters to refine glutamatergic excitatory signals (Franke & Baden, 2017; Werblin, 2011; C. …
The Drosophila Neuroblasts: A Model System For Human Ribosomopathies, Sonu Shrestha Baral
The Drosophila Neuroblasts: A Model System For Human Ribosomopathies, Sonu Shrestha Baral
LSU Doctoral Dissertations
This dissertation describes the use of Drosophila neuroblasts (NBs) to model human ribosomopathies; the overall goal is to understand why specific stem cell and progenitor cell populations are the primary targets in nucleolar stress as seen in the ribosomopathies. Chapter 1 provides an overview of relevant literature. Chapter 2 describes nucleolar stress in Drosophila neuroblasts as a model for human ribosomopathies. For this, we induce nucleolar stress by using the UAS-GAL4 system to express RNAi that depletes Nopp140 transcripts, and we also employ homozygous, CRISPR-Cas9-generated Nopp140 gene disruptions with a systemic null phenotype (Nopp140-/-). Embryonic lethality was observed …
Ck2 Negatively Regulates 5-Ht4 Receptor Signaling In The Prefrontal Cortex And Mediates Depression-Like Behaviors, Julia Castello Saval
Ck2 Negatively Regulates 5-Ht4 Receptor Signaling In The Prefrontal Cortex And Mediates Depression-Like Behaviors, Julia Castello Saval
Dissertations, Theses, and Capstone Projects
The serotonergic system has been the major candidate in the pathophysiology of mood related disorders such as anxiety and major depressive disorder (MDD). Unfortunately, current antidepressant drugs are ineffective in 50% of the population and require chronic administration for a period of 3-6 weeks before the onset of therapeutic response. 5-HT4 receptor (5-HT4R) agonists have emerged as potential candidates for fast antidepressant action, since an antidepressant response can be achieved after 3 days of pharmacological administration in rodents.
This dissertation aims to investigate the role of casein kinase 2 (CK2) as a regulator of 5-HT4R expression …
The Role Of H3k4 Methyltransferases In Drosophila Memory, Nicholas Raun
The Role Of H3k4 Methyltransferases In Drosophila Memory, Nicholas Raun
Electronic Thesis and Dissertation Repository
Gene transcription required for long-term memory requires the modification of histones. However, there are still many uncertainties about the identity and spatial expression of genes regulated by histone modifications during memory related processes. In this project I examined the role of Drosophila melanogaster methyltransferases Set1 and trx in courtship memory. Genetic knockdown of Set1 and trx in the mushroom body (MB) revealed that Set1 was necessary for short- and long-term memory, while trx was only required for long-term memory. Transcriptional profiling of MBs following trx-knockdown revealed expression changes in MB-enriched genes and genes involved in RNA processing. Among the …
Biochemical Approaches For The Diagnosis And Treatment Of Lafora Disease, Mary Kathryn Brewer
Biochemical Approaches For The Diagnosis And Treatment Of Lafora Disease, Mary Kathryn Brewer
Theses and Dissertations--Molecular and Cellular Biochemistry
Glycogen is the sole carbohydrate storage molecule found in mammalian cells and plays an important role in cellular metabolism in nearly all tissues, including the brain. Defects in glycogen metabolism underlie the glycogen storage diseases (GSDs), genetic disorders with variable clinical phenotypes depending on the mutation type and affected gene(s). Lafora disease (LD) is a fatal form of progressive myoclonus epilepsy and a non-classical GSD. LD typically manifests in adolescence with tonic-clonic seizures, myoclonus, and a rapid, insidious progression. Patients experience increasingly severe and frequent epileptic episodes, loss of speech and muscular control, disinhibited dementia, and severe cognitive decline; death …
Mutations Of Fus Cause Aggregation Of Rna Binding Proteins, Disruptions In Protein Synthesis, And Dysregulation Of Nonsense Mediated Decay, Marisa Elizabeth Kamelgarn
Mutations Of Fus Cause Aggregation Of Rna Binding Proteins, Disruptions In Protein Synthesis, And Dysregulation Of Nonsense Mediated Decay, Marisa Elizabeth Kamelgarn
Theses and Dissertations--Toxicology and Cancer Biology
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by motor neuron death and subsequent muscle atrophy. Approximately 15% of ALS cases are inheritable, and mutations in the Fused in Sarcoma (FUS) gene contribute to approximately 5% of these cases, as well as about 2% of sporadic cases. FUS performs a diverse set of cellular functions, including being a major regulator of RNA metabolism. FUS undergoes liquid- liquid phase transition in vitro, allowing for its participation in stress granules and RNA transport granules. Phase transition also contributes to the formation of cytoplasmic inclusions found in the …