Method Article

In Vitro Induction of Neuromuscular Junctions from Engineered Human-Induced Pluripotent Stem Cells

July 8th, 2025

In This Article

Abstract

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Source:Lin, C. et al., In vitro Neuromuscular Junction Induced from Human Induced Pluripotent Stem Cells. J. Vis. Exp.(2020).

This experiment details a method for generating neuromuscular junctions from human-induced pluripotent stem cells. It uses a staged differentiation process to promote myotube and motor neuron development and synapse formation, yielding functional neuromuscular junctions.

Protocol

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1. Preparation of extracellular matrix (ECM)-coated plates

  1. Dilute ECM (see Table of Materials) with ice cold 1x PBS to a final concentration of 2%
  2. Add 1 mL of ECM to 49 mL of 1x PBS in a 50 mL plastic tube. Mix well by pipetting up and down several times.
  3. Place 1 coverslip into each well of a 6-well plate. Add 1.5 mL of 2% ECM into each well.
  4. Incubate the well plate with 2% ECM for 2 h at 37 °C.
  5. Aspirate ECM from the well plate and store the plate at 4 °C before use.

2. Differentiation of iPSCs toward NMJ

  1. Seed 4 x 105 of iPSCs per well on the 6-well plate prepared in section 1. Make sure to seed the cells on the coverslip pre-deposited in the well.
    NOTE: In this study, the 201B7MYOD iPS cell line was used.
    1. Remove the medium from the iPSCs on the 6 cm culture dish and wash the iPSCs once with 1x PBS.
    2. Add 1 mL of cell detachment solution to the dish and incubate for 10 min at 37 °C.
    3. Add 3 mL of primate embryonic stem (ES) cell medium to the dish and gently pipette 3 times.
    4. Collect the supernatant, which contains detached iPSCs, into a 50 mL plastic tube and centrifuge at 160 x g at 4 °C for 5 min.
    5. Carefully aspirate the supernatant, resuspend the iPSCs in 3 mL primate ES cell medium with 10 µM Y27632, and count the cell number using a hemocytometer.
    6. Dilute the iPSCs with primate ES cell medium and 10 µM Y27632 to a concentration of 2 x 105 cells/mL. Add 2 mL of iPSCs on the coverslip pre-deposited in the well described in section 1.

3. Induction of the iPSCs to NMJ

  1. On day 1, 24 h after seeding the iPSCs to the 6-well plate, remove the culture medium and replace it with 2 mL of fresh primate ES cell medium containing 1 µg/mL doxycycline to each well.
  2. Change the medium with 2 mL of myogenic differentiation medium (MDM) (Table 1) containing 1 µg/mL doxycycline (final concentration) to each well. Refresh the medium every day from day 2 to day 10.
  3. From day 11, switch the medium to 2 mL of NMJ medium (Table 1) to each well. Refresh the medium every 3‒4 days thereafter until day 30.
  4. Observe the differentiated NMJ by phase inverted microscopy on day 30. The NMJ can be used for the following analysis.

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Results

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Table 1: Formula of medium.

Myogenic differentiation medium (MDM)MEM-alpha500mL
100mM 2-ME1117μL
Doxycycline1μg/mL
KSR56mL (final to 10%)
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Disclosures

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No conflicts of interest declared.

Materials

List of materials used in this article
NameCompanyCatalog NumberComments
Medium, growth factors and reagents
2-mercaptoethanolNacalai tesque21418-42
B27, GibcoGibco12587-001
BDNFR & D Systems248-BD
Cell detachment solution, AccumaxSTEMCELL Technologies#07921
Critical point dryerHitachiES-2030
DoxycyclineTakara631311
ECM, Matrigel (growth factor reduced)Corning356230
GDNFR & D Systems212-GD
Gelation, IP gelGeno StaffPG20-1
Ions coaterJEOLJEC3000FC
iPSC medium, mTeSRSTEMCELL Technologies85850
KnockOut SR (KSR)Gibco10828-028
live cell microscopyNikonEclipse Ti microscope
MEM-alphaGibco12571-071
N2, GibcoGibco17502-048
Neurobasal mediumGibco21103-049
NT3R & D Systems267-N3
Primate ES cell mediumReproCellRCHEMD001
SEMHitachiS-4700
TEMHitachiH7650
Y27632Wako Chemicals GmbH253-00513

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Tags

Human iPSCsMYOD InductionMyotube FormationMotor Neuron DifferentiationNeuromuscular JunctionDoxycycline TreatmentROCK InhibitorECM CoatingCell DifferentiationSynapse Formation

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