The neuromuscular junction (NMJ) is a specialized synapse that bridges the motor neuron and the skeletal muscle fiber and is crucial for conversion of electrical impulses originating in the motor neuron to action potentials in the muscle fiber.

There are currently no functional neuromuscular junction (hNMJ) systems composed of human cells that could be used for drug evaluations or toxicity testing in vitro. These systems are needed to evaluate NMJs for diseases such as amyotrophic lateral sclerosis, spinal muscular atrophy or other neurode …

2021年9月24日 · In the neuromuscular system, signal transmission from motor neurons (MNs) to innervated muscle fibers is crucial for their synaptic function, viability, and maintenance. In order to better understand human neuromuscular junction (hNMJ) functionality, it is important to develop on-a-chip devices with human ce

2021年9月1日 · Here we describe a standardized method for engineering an isogenic, patient specific human neuromuscular junction (NMJ) that allows for automated quantification of NMJ function to diagnose disease using a small sample of …

The knockdown caused degenerated features in the NMJ and muscle, and the NMJ showed functional defects consistent with SMA when stimulated chemically or optogenetically. We propose our hNMJ model for investigating the physiology and pathology of hNMJs and for evaluating related therapeutic molecules. Results

2020年12月1日 · Here, we describe current models for neural, tendinous, and vascular connections in hSkMTs and their limitations. The NMJ is a synaptic connection wherein acetylcholine (ACh) release from the MN triggers an action potential in skeletal muscle, resulting in voluntary movement.

In order to better understand human neuromuscular junction (hNMJ) functionality, we developed on-a-chip devices to grow hNMJs on a specific electrode pattern to stimulate pre-synaptic axons and record post-synaptic muscle activity.

2017年11月28日 · Here, we combined morphological techniques, super-resolution imaging, and proteomic profiling to reveal the detailed cellular and molecular architecture of the human NMJ. Human NMJs were significantly smaller, less complex, and more fragmented than mouse NMJs.

Human NMJs are among the smallest known and release the smallest number of transmitter “quanta”. By contrast, they have the most deeply infolded postsynaptic membranes, which help to amplify transmitter action. The same structural features that distinguish human NMJs make them particularly susceptible to pathological processes.

There are currently no functional neuromuscular junction (hNMJ) systems composed of human cells that could be used for drug evaluations or toxicity testing in vitro. These systems are needed to evaluate NMJs for diseases such as amyotrophic lateral sclerosis, spinal muscular atrophy or other neurodegenerative diseases or injury states.