TY - JOUR
T1 - Exploring the interaction of drosophila TDP-43 and the type ii voltage-gated calcium channel, cacophony, in regulating motor function and behavior
AU - Lembke, Kayly M.
AU - Morton, David B.
N1 - Publisher Copyright:
© The Author(s) 2017.
PY - 2017/1/1
Y1 - 2017/1/1
N2 - 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, displayed by tbph mutants, are rescued by genetically restoring a voltage-gated calcium channel to either motor neurons or just a single pair of neurons in the brain. If these effects are mirrored in human TDP-43 proteinopathies, these observations could open new avenues to investigate alternative therapeutic targets for these neurodegenerative diseases.
AB - 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, displayed by tbph mutants, are rescued by genetically restoring a voltage-gated calcium channel to either motor neurons or just a single pair of neurons in the brain. If these effects are mirrored in human TDP-43 proteinopathies, these observations could open new avenues to investigate alternative therapeutic targets for these neurodegenerative diseases.
KW - ALS
KW - Amyotrophic lateral sclerosis
KW - Drosophila melanogaster
KW - Neurodegeneration
KW - Voltage-gated ion channel
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U2 - 10.1177/1179069517740892
DO - 10.1177/1179069517740892
M3 - Article
AN - SCOPUS:85041905270
SN - 1179-0695
VL - 11
JO - Journal of Experimental Neuroscience
JF - Journal of Experimental Neuroscience
ER -