Open Access. Powered by Scholars. Published by Universities.®

Life Sciences Commons

Open Access. Powered by Scholars. Published by Universities.®

George Fox University

Series

Amyotrophic lateral sclerosis

Articles 1 - 2 of 2

Full-Text Articles in Life Sciences

Exploring The Interaction Of Drosophila Tdp-43 And The Type Ii Voltage-Gated Calcium Channel, Cacophony, In Regulating Motor Function And Behavior, Kayly M. Lembke, David B. Morton Jan 2017

Exploring The Interaction Of Drosophila Tdp-43 And The Type Ii Voltage-Gated Calcium Channel, Cacophony, In Regulating Motor Function And Behavior, Kayly M. Lembke, David B. Morton

Faculty Publications - Department of Biological & Molecular Science

Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neurodegenerative disease. The cause of the disease remains obscure, and as such there is no effective treatment or cure. Amyotrophic lateral sclerosis and other neurodegenerative diseases are frequently characterized by dysfunction of the RNA-binding protein, TDP-43. Using model systems to understand the mechanisms underlying TDP-43 dysfunction should accelerate identification of therapeutic targets. A recent report has shown that motor defects caused by the deletion of the Drosophila TDP-43 ortholog, tbph, are not driven by changes in the physiology at the neuromuscular junction. Rather, defective motor burst rhythmicity and coordination, …


Restoration Of Motor Defects Caused By Loss Of Drosophila Tdp-43 By Expression Of The Voltage-Gated Calcium Channel, Cacophony, In Central Neurons, Kayly M. Lembke, David B. Norton, Charles Scudder Jan 2017

Restoration Of Motor Defects Caused By Loss Of Drosophila Tdp-43 By Expression Of The Voltage-Gated Calcium Channel, Cacophony, In Central Neurons, Kayly M. Lembke, David B. Norton, Charles Scudder

Faculty Publications - Department of Biological & Molecular Science

Defects in the RNA-binding protein, TDP-43, are known to cause a variety of neurodegenerative diseases, including amyotrophic lateral sclerosis and frontotemporal lobar dementia. A variety of experimental systems have shown that neurons are sensitive to TDP-43 expression levels, yet the specific functional defects resulting from TDP-43 dysregulation have not been well described. Using the Drosophila TDP-43 ortholog TBPH, we previously showed that TBPH-null animals display locomotion defects as third instar larvae. Furthermore, loss of TBPH caused a reduction in cacophony, a Type II voltage-gated calcium channel, expression and that genetically restoring cacophony in motor neurons in TBPH mutant animals was …