A Novel In Vitro Primary Culture Model of the Lower Motor Neuron–Neuromuscular Junction Circuit

2015 ◽  
pp. 181-193 ◽  
Author(s):  
Katherine A. Southam ◽  
Anna E. King ◽  
Catherine A. Blizzard ◽  
Graeme H. McCormack ◽  
Tracey C. Dickson
Keyword(s):  
Neuromuscular Junction ◽  
Motor Neuron ◽  
Primary Culture ◽  
Lower Motor Neuron ◽  
Culture Model

Microfluidic primary culture model of the lower motor neuron–neuromuscular junction circuit

2013 ◽  
Vol 218 (2) ◽  
pp. 164-169 ◽  
Author(s):  
Katherine A. Southam ◽  
Anna E. King ◽  
Catherine A. Blizzard ◽  
Graeme H. McCormack ◽  
Tracey C. Dickson
Keyword(s):  
Neuromuscular Junction ◽  
Motor Neuron ◽  
Primary Culture ◽  
Lower Motor Neuron ◽  
Culture Model

Weakness

2018 ◽  
pp. 207-238
Author(s):  
Andrea C. Adams
Keyword(s):  
Nervous System ◽  
Neuromuscular Junction ◽  
Motor Neuron ◽  
Motor System ◽  
Lower Motor Neuron ◽  
Diagnostic Challenge ◽  
Common Complaint ◽  
Motor Nerves

Weakness is a common complaint. Most patients use the term weakness to imply fatigue, general illness, or myalgias. Determining whether a patient has actual neuromuscular weakness can be a diagnostic challenge. Disease of the motor system can occur at all levels of the nervous system. This chapter considers disorders of the lower motor neuron, including disorders of muscle (myopathies), the neuromuscular junction, and motor nerves.

scholarly journals Neuromuscular junction maturation defects precede impaired lower motor neuron connectivity in Charcot-Marie-Tooth type 2D mice

2013 ◽  
Vol 23 (10) ◽  
pp. 2639-2650 ◽  
Author(s):  
J. N. Sleigh ◽  
S. J. Grice ◽  
R. W. Burgess ◽  
K. Talbot ◽  
M. Z. Cader
Keyword(s):  
Neuromuscular Junction ◽  
Motor Neuron ◽  
Lower Motor Neuron ◽  
Charcot Marie Tooth ◽  
Tooth Type

scholarly journals Modelling functional human neuromuscular junctions in a differentially-perturbable microfluidic environment, validated through recombinant monosynaptic pseudotyped ΔG-rabies virus tracing

2019 ◽  
Author(s):  
Ulrich Stefan Bauer ◽  
Rosanne van de Wijdeven ◽  
Rajeevkumar Nair Raveendran ◽  
Vegard Fiskum ◽  
Clifford Kentros ◽  
...  
Keyword(s):  
Neuromuscular Junction ◽  
Motor Neuron ◽  
Rabies Virus ◽  
Motor Neurons ◽  
Microfluidic Chip ◽  
Neuromuscular Junctions ◽  
Patient Specific ◽  
Human Myotubes ◽  
Virus Tracing

AbstractCompartmentalized microfluidic culture systems provide new perspectives in in vitro disease modelling as they enable co-culture of different relevant cell types in interconnected but fluidically isolated microenvironments. Such systems are thus particularly interesting in the context of in vitro modelling of mechanistic aspects of neurodegenerative diseases such as amyotrophic lateral sclerosis, which progressively affect the function of neuromuscular junctions, as they enable the co-culture of motor neurons and muscle cells in separate, but interconnected compartments. In combination with cell reprogramming technologies for the generation of human (including patient-specific) motor neurons, microfluidic platforms can thus become important research tools in preclinical studies. In this study, we present the application of a microfluidic chip with a differentially-perturbable microenvironment as a platform for establishing functional neuromuscular junctions using human induced pluripotent stem cell derived motor neurons and human myotubes. As a novel approach, we demonstrate the functionality of the platform using a designer pseudotyped ΔG-rabies virus for retrograde monosynaptic tracing.Graphical abstractFunctional neuromuscular junction in a microfluidic chip(a) Overview of microfluidic chip. Human iPS cell-derived motor neuron aggregates (spheroids indicated by black arrows) are seeded in the three lateral compartments of the chip, while human myotubes (white arrows) are seeded in the middle compartment.(b) Directed connectivity and retrograde virus tracing. Outgrowing axons (yellow arrow) from the motor neuron aggregate enter the directional axon tunnels (grey rectangles) and form connections with the myotubes (white arrow) within the opposite compartment. Addition of a designer monosynaptic pseudotyped ΔG-rabies virus to the myotube compartment, infects the myotubes (green) expressing an exogenous receptor (TVA) and rabies glycoprotein (G), subsequently making infectious viruses that are retrogradely transported through the motor neuron axons (green arrow) back to the neuronal cell bodies within the aggregate, validating neuromuscular junction functionality.

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