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Full-Text Articles in Nervous System Diseases
Novel Therapeutic Approaches For Juvenile Neuronal Ceroid Lipofuscinosis (Cln3), Megan Elizabeth Bosch
Novel Therapeutic Approaches For Juvenile Neuronal Ceroid Lipofuscinosis (Cln3), Megan Elizabeth Bosch
Theses & Dissertations
Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) is a lysosomal storage disease caused by autosomal recessive mutations in CLN3. Neuronal loss is thought to occur via glutamate excitotoxicity; however, little is known about neuron-astrocyte glutamate regulation in JNCL. We discovered that Cln3Δex7/8 astrocytes have significantly lower basal spontaneous Ca2+ oscillations and decreased responses to glutamate, indicating a disrupted signaling network. Cln3Δex7/8 astrocytes also displayed significantly lower basal mitochondrial respiration and ATP production, suggesting impaired metabolic functions. Concurrent with diminished astrocyte metabolism and Ca2+ signaling, Cln3Δex7/8 neurons were hyper-responsive to glutamate stimulation. These studies suggest that CLN3 …
Evaluation Of Immune-Modulating Therapies For Parkinson's Disease, Katherine E. Olson
Evaluation Of Immune-Modulating Therapies For Parkinson's Disease, Katherine E. Olson
Theses & Dissertations
Parkinson’s disease (PD) is the second most common neurodegenerative
disorder, second only to Alzheimer’s disease (AD). It is characterized by a
progressive loss of dopaminergic neurons along the nigrostriatal axis and the
formation of proteinaceous inclusions of alpha-synuclein (α-syn). Secondary to
the loss of dopaminergic neurons is a progression in motor and non-motor
symptoms. Motor symptoms are characterized by slowness in movement,
stiffness and tremor. Non-motor symptoms include depression, constipation,
sleep abnormalities and loss of sense of smell. The cause of disease remains
incompletely understood. However, age, genetics, environment, viral infection,
and interplay between the innate and adaptive immune system …