scholarly journals C 9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits

2015 ◽  
Vol 78 (3) ◽  
pp. 426-438 ◽  
Author(s):  
Max Koppers ◽  
Anna M. Blokhuis ◽  
Henk‐Jan Westeneng ◽  
Margo L. Terpstra ◽  
Caroline A. C. Zundel ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Deficits ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration

scholarly journals Circuit dysfunction in SOD1-ALS model first detected in sensory feedback prior to motor neuron degeneration is alleviated by BMP signaling

2018 ◽  
Author(s):  
Aaron Held ◽  
Paxton Major ◽  
Asli Sahin ◽  
Robert Reenan ◽  
Diane Lipscombe ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Function ◽  
Motor Neurons ◽  
Sensory Feedback ◽  
Early Stage ◽  
Bmp Signaling ◽  
Motor Deficits ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Motor Circuit

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease whose origin and underlying cellular defects are still not fully understood. While motor neuron degeneration is the signature feature of ALS, it is not yet clear if motor neurons, or other cells of the motor circuit, are the site of disease initiation. To better understand the contribution of multiple cell types in ALS, we made use of a Drosophila Sod1G85R knock-in model, in which all cells harbor the disease allele. End-stage dSod1G85R animals exhibit severe motor deficits with clear degeneration of motor neurons. Interestingly, earlier in dSod1G85R larvae motor function is also compromised, but their motor neurons exhibit only subtle morphological and electrophysiological changes, that are unlikely to cause the observed decrease in locomotion. We analyzed the intact motor circuit and identified a defect in sensory feedback that likely accounts for the altered motor activity of dSod1G85R. Furthermore, we found that the cell-autonomous activation of BMP signaling in proprioceptor sensory neurons that relay the contractile status of muscles back to the central nerve cord, is able to completely rescue early stage motor defects and partially rescue late stage motor function to extend lifespan. Identifying a defect in sensory feedback as a potential initiating event in ALS motor dysfunction, coupled with the ability of modified proprioceptors to alleviate such motor deficits, underscores the critical role that non-motor neurons play in disease progression and highlights their potential as a site to identify early-stage ALS biomarkers and for therapeutic intervention.

Cytoplasmic inclusions in spinal neurons of the motor neuron degeneration (Mnd) mouse

1993 ◽  
Vol 116 (1) ◽  
pp. 59-66 ◽  
Author(s):  
J.E. Mazurkiewicz ◽  
L.M. Callahan ◽  
M. Swash ◽  
J.E. Martin ◽  
A. Messer
Keyword(s):  
Motor Neuron ◽  
Spinal Neurons ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Cytoplasmic Inclusions
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