Deletion of a specific exon in the voltage-gated calcium channel gene disrupts locomotion in larvae.

TAR DNA-binding protein 43 (TDP-43) is an RNA-binding protein that regulates transcription, translation and alternative splicing of mRNA. We have shown previously that null mutations of the ortholog, (), causes severe locomotion defects in larvae that are mediated by a reduction in the expression of a type II voltage-gated calcium channel, (). We also showed that TDP-43 regulates the inclusion of alternatively spliced exons of ; mutants showed significantly increased expression of isoforms lacking exon 7, a particularly notable finding as only one out of the 15 predicted isoforms lacks exon 7.

Exo70 Is Essential for Neurite Extension and Survival under Thermal Stress.

The octomeric exocyst complex governs the final step of exocytosis in both plants and animals. Its roles, however, extend beyond exocytosis and include organelle biogenesis, ciliogenesis, cell migration, and cell growth. Exo70 is a conserved component of the exocyst whose function in is unclear.

Exploring the Interaction of TDP-43 and the Type II Voltage-Gated Calcium Channel, , in Regulating Motor Function and Behavior.

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.

Restoration of Motor Defects Caused by Loss of TDP-43 by Expression of the Voltage-Gated Calcium Channel, , in Central Neurons.

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 TDP-43 ortholog TBPH, we previously showed that TBPH-null animals display locomotion defects as third instar larvae.