TY  - GEN
N2  - Amyotrophic Lateral Sclerosis is the most common adult onset motor neurodegenerative disease. The etiology of the disease remains obscure, and as such there is no effective treatment or cure. TDP-43 is a nuclear RNA and DNA binding protein associated with 96% of ALS patient pathology. Tissue samples taken post-mortem from patients show a loss of nuclear localization and the presence of TDP-43 in cytoplasmic inclusions in the cell body. Whether the pathogenesis of the disease is driven by loss of nuclear function, gain of cytotoxicity with the formation of the cytoplasmic inclusions, or some other yet unidentified cause is not known. The goal of the research presented here was to understand the downstream effects of loss of nuclear TDP-43 function on motor behavior and physiology, using Drosophila melanogaster as a model. The results I present here show that behavioral motor defects associated with loss of Drosophila TDP-43 are driven by the loss of the voltage gated calcium channel cacophony, not at the neuromuscular junction, but rather in cells located centrally in the protocerebrum of the brain.
DO  - 10.6083/m4vt1r61
DO  - DOI
AB  - Amyotrophic Lateral Sclerosis is the most common adult onset motor neurodegenerative disease. The etiology of the disease remains obscure, and as such there is no effective treatment or cure. TDP-43 is a nuclear RNA and DNA binding protein associated with 96% of ALS patient pathology. Tissue samples taken post-mortem from patients show a loss of nuclear localization and the presence of TDP-43 in cytoplasmic inclusions in the cell body. Whether the pathogenesis of the disease is driven by loss of nuclear function, gain of cytotoxicity with the formation of the cytoplasmic inclusions, or some other yet unidentified cause is not known. The goal of the research presented here was to understand the downstream effects of loss of nuclear TDP-43 function on motor behavior and physiology, using Drosophila melanogaster as a model. The results I present here show that behavioral motor defects associated with loss of Drosophila TDP-43 are driven by the loss of the voltage gated calcium channel cacophony, not at the neuromuscular junction, but rather in cells located centrally in the protocerebrum of the brain.
T1  - The ALS-associated protein, TDP-43, functions to regulate drosophila larval motor behavior and physiology in a cacophony-dependent manner
DA  - 2017
AU  - Lembke, Kayly M.
L1  - https://digitalcollections.ohsu.edu/record/7672/files/Lembke.Kayly.2017.pdf
PB  - Oregon Health and Science University
PY  - 2017
ID  - 7672
L4  - https://digitalcollections.ohsu.edu/record/7672/files/Lembke.Kayly.2017.pdf
KW  - Calcium Channels
KW  - Drosophila
KW  - Amyotrophic Lateral Sclerosis
KW  - Motor Neurons
TI  - The ALS-associated protein, TDP-43, functions to regulate drosophila larval motor behavior and physiology in a cacophony-dependent manner
Y1  - 2017
L2  - https://digitalcollections.ohsu.edu/record/7672/files/Lembke.Kayly.2017.pdf
LK  - https://digitalcollections.ohsu.edu/record/7672/files/Lembke.Kayly.2017.pdf
UR  - https://digitalcollections.ohsu.edu/record/7672/files/Lembke.Kayly.2017.pdf
ER  -