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Full-Text Articles in Life Sciences
Modeling Als-Associated Matrin-3 Toxicity In Yeast, Widad El-Zein
Modeling Als-Associated Matrin-3 Toxicity In Yeast, Widad El-Zein
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ALS is a neurodegenerative disease characterized by degeneration of upper and lower motor neurons in the brain and spinal cord leading to progressive paralysis and ultimately death. Perturbations in RNA metabolism and RNA binding proteins have emerged as underlying defects in ALS pathogenesis. Matrin-3 is a multifunctional RNA binding protein that has been linked to familial and sporadic ALS. Matrin-3 is normally found in the nucleus, but mutations in the gene cause mislocalization of the protein from the nucleus into the cytoplasm of neuronal cells where it forms protein aggregates. In this study, we show that over-expressing human MATR3 in …
Protocol Development And Optimization For Rnls Mouse Characteristic Assessment, Hasan Farid
Protocol Development And Optimization For Rnls Mouse Characteristic Assessment, Hasan Farid
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Protocol development and optimization are vital in the scientific method process. By having accurate protocols, one can properly assess the characteristics of their animal model for any given experiment. One animal newly adopted in our lab was the novel regulatable nuclear localization sequence (rNLS) mouse model. This novel mouse model displays symptoms of Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal dementia (FTD), after the accumulation of the hTDP-43 (TAR DNA-binding protein 43) aggregate in the central nervous system. The expression of this protein occurs after the removal of deoxycycline from the mouse’s food source. Once the removal of the drug, this …
Investigating The Role Of An Sk Channel Activator On Survival And Motor Function In The Sod1-G93a, Als Mouse Model, Matthew Thomas Dancy
Investigating The Role Of An Sk Channel Activator On Survival And Motor Function In The Sod1-G93a, Als Mouse Model, Matthew Thomas Dancy
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Amyotrophic Lateral Sclerosis (ALS) is a fatal, adult-onset progressive degenerative motor neuron disease that is characterized by muscle atrophy and weakness due to the loss of upper and lower motor neurons. Average survival time for individuals diagnosed with the disease is three to five years; currently there is no cure and only one drug approved by the Food and Administration (FDA). Scientists have proposed various theories in order to solve the mystery which surrounds ALS. One of these theories hypothesizes how hyperexcitability and excitotoxicity leads to the death of motor neurons. In this study, we will address ways of combatting …
Determining Protein-Protein Interactions Of Als-Associated Sod1, Leah A. Shurte
Determining Protein-Protein Interactions Of Als-Associated Sod1, Leah A. Shurte
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Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disorder that occurs due to the death of motor neurons and leads to paralysis and death within three to five years after symptoms present (Byrne et al., 2013). Superoxide Dismutase 1 (SOD1) was first identified to be associated with ALS in 1993. The objective of this study is to determine which proteins interact with wild type and mutant SOD1 and find any similarities or differences between them. ALS is attributed to a gain of toxicity, therefore abnormal protein interactions in mutant SOD1 are important. The results of this study will provide insight on …
Sk Channel Clustering In Sod1-G93a Motoneurons, Saihari Shekar Dukkipati
Sk Channel Clustering In Sod1-G93a Motoneurons, Saihari Shekar Dukkipati
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Amyotrophic lateral sclerosis (ALS) is a devastating neuromuscular disease that currently has no cure and extremely limited treatment options. The specific mechanisms that underlie motoneuron degeneration and death, which are classical features of this disease, are mostly unknown. This thesis tests the hypothesis that small-conductance calcium-activated potassium channels (SK) may be downregulated in ALS motoneurons, as suggested by computational modelling. SK channel expression was measured in spinal alpha-motoneuron cell bodies or somata of wildtype (WT) and mutant (mt) SOD1-G93A mice, a transgenic animal model of ALS. Quantitative immunohistochemical analysis of the developmental expression of SK channel isoforms SK2 and SK3 …
Fission Yeast As A Model Organism For Fus-Dependent Ycytotoxicity In Amyotrophic Lateral Sclerosis, Alan J. Cone
Fission Yeast As A Model Organism For Fus-Dependent Ycytotoxicity In Amyotrophic Lateral Sclerosis, Alan J. Cone
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Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative motor neuron disease that causes progressive paralysis and death by asphyxiation. There is no cure or effective treatment; however, previous research has identified several genes that appear related to the pathology of ALS. When mutated, these genes result in proteins that gain toxic functions and disrupt normal cellular processes. Fused in Sarcoma (hFUS) is a human transcription factor in the nucleus that binds to DNA and RNA. Mutations in hFUS are associated with both familial and sporadic cases of ALS, frontotemporal lobar degeneration (FTLD), and cancer. In ALS and FTLD, hFUS is mislocalized …