scholarly journals Generation of Human Motor Units with Functional Neuromuscular Junctions in Microfluidic Devices

10.3791/62959 ◽  
2021 ◽  
Author(s):  
Katarina Stoklund Dittlau ◽  
Emily N. Krasnow ◽  
Laura Fumagalli ◽  
Tijs Vandoorne ◽  
Pieter Baatsen ◽  
...  
Keyword(s):  
Neuromuscular Junctions ◽  
Microfluidic Devices ◽  
Motor Units ◽  
Human Motor ◽  
Human Motor Units

scholarly journals Human motor units in microfluidic devices are impaired by FUS mutations and improved by HDAC6 inhibition

2021 ◽  
Author(s):  
Katarina Stoklund Dittlau ◽  
Emily N. Krasnow ◽  
Laura Fumagalli ◽  
Tijs Vandoorne ◽  
Pieter Baatsen ◽  
...  
Keyword(s):  
Microfluidic Devices ◽  
Motor Units ◽  
Human Motor ◽  
Human Motor Units

scholarly journals Human motor units in microfluidic devices are impaired by FUS mutations and improved by HDAC6 inhibition

2020 ◽  
Author(s):  
Katarina Stoklund Dittlau ◽  
Emily N. Krasnow ◽  
Laura Fumagalli ◽  
Tijs Vandoorne ◽  
Pieter Baatsen ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Neurite Outgrowth ◽  
Motor Neurons ◽  
Neuromuscular Junctions ◽  
Induced Pluripotent Stem Cell ◽  
Microfluidic Devices ◽  
Motor Units ◽  
Human Motor ◽  
Induced Pluripotent

AbstractNeuromuscular junctions (NMJs) ensure proper communication between motor neurons and muscle through the release of neurotransmitters. In motor neuron disorders, such as amyotrophic lateral sclerosis (ALS), NMJs degenerate resulting in muscle atrophy, paralysis and respiratory failure. The aim of this study was to establish a versatile and reproducible in vitro model of a human motor unit to study the effect of ALS-causing mutations. Therefore, we generated a co-culture of human induced pluripotent stem cell-derived motor neurons and human primary mesoangioblast-derived myotubes in microfluidic devices. A chemotactic and volumetric gradient facilitated the growth of motor neuron neurites through microgrooves resulting in the interaction with myotubes and the formation of NMJs. We observed that ALS-causing FUS mutations resulted in a reduced neurite outgrowth and in a decreased NMJ number. Interestingly, the selective HDAC6 inhibitor, Tubastatin A, improved the neurite outgrowth and the NMJ morphology of FUS-ALS co-cultures, further prompting HDAC6 inhibition as a potential therapeutic strategy for ALS.

Discharge patterns in human motor units during fatiguing arm movements

1998 ◽  
Vol 85 (5) ◽  
pp. 1684-1692 ◽  
Author(s):  
L. Griffin ◽  
S. J. Garland ◽  
T. Ivanova
Keyword(s):  
Voluntary Movement ◽  
Motor Units ◽  
Constant Load ◽  
Short Isis ◽  
Interspike Intervals ◽  
Triceps Brachii ◽  
Movement Initiation ◽  
Human Motor ◽  
Discharge Patterns ◽  
Human Motor Units

The purpose of this study was to determine whether short interspike intervals (ISIs of <20 ms) would occur naturally during voluntary movement and would increase in number with fatigue. Thirty-four triceps brachii motor units from nine subjects were assessed during a fatigue task consisting of fifty extension and fifty flexion elbow movements against a constant-load opposing extension. Nineteen motor units were recorded from the beginning of the fatigue task; the number of short ISIs was 7.1 ± 4.1% of the total number of ISIs in the first one-third of the task (unfatigued state). This value increased to 11.8 ± 5.9% for the last one-third of the task (fatigued state). Fifteen motor units were recruited during the fatigue task and discharged, with 16.4 ± 6.0% of short ISIs in the fatigued state. For all motor units, the number of short ISIs was positively correlated ( r 2 = 0.85) with the recruitment threshold torque. Short ISIs occurred most frequently at movement initiation but also occurred throughout the movement. These results document the presence of short ISIs during voluntary movement and their increase in number during fatigue.

scholarly journals Non-invasive imaging of single human motor units

2020 ◽  
Vol 131 (6) ◽  
pp. 1399-1406 ◽  
Author(s):  
Matthew G. Birkbeck ◽  
Linda Heskamp ◽  
Ian S. Schofield ◽  
Andrew M. Blamire ◽  
Roger G. Whittaker
Keyword(s):  
Motor Units ◽  
Non Invasive ◽  
Human Motor ◽  
Human Motor Units

Ischemia sensitivity and motoneuron afterhyperpolarization in human motor units

2004 ◽  
Vol 30 (2) ◽  
pp. 195-201 ◽  
Author(s):  
E. Roderich Gossen ◽  
Tanya D. Ivanova ◽  
S. Jayne Garland
Keyword(s):  
Motor Units ◽  
Human Motor ◽  
Human Motor Units

Firing rates of human motor units in partially denervated muscle

Neurology ◽  
1978 ◽  
Vol 28 (12) ◽  
pp. 1241-1241 ◽  
Author(s):  
R. G. Miller ◽  
M. Sherratt
Keyword(s):  
Motor Units ◽  
Denervated Muscle ◽  
Firing Rates ◽  
Human Motor ◽  
Human Motor Units

Time-dependent structure in the discharge rate of human motor units

2002 ◽  
Vol 113 (8) ◽  
pp. 1325-1338 ◽  
Author(s):  
David E. Vaillancourt ◽  
Lars Larsson ◽  
Karl M. Newell
Keyword(s):  
Discharge Rate ◽  
Motor Units ◽  
Time Dependent ◽  
Human Motor ◽  
Human Motor Units
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