Science Advances

Supplementary Materials

The PDF file includes:

  • Fig. S1. Characterization and differentiation of iPS-derived skeletal myoblasts in a monolayer culture.
  • Fig. S2. Comparison between a mouse muscle fiber bundle of C2C12 and a human muscle fiber bundle of iPS-derived skeletal muscle cells.
  • Fig. S3. Muscle contraction and synchronization by chemical stimulation.
  • Fig. S4. Glutamic acid treatment and electrical stimulation and TTX treatment to the motor unit model.
  • Fig. S5. Characterization of iPS-derived MN from a sporadic ALS donor.
  • Fig. S6. Genotyping of ALS-iPS–derived MN and ES-derived MN.
  • Fig. S7. Morphogenesis of MN spheroids derived from a patient with ALS in 2D culture.
  • Fig. S8. Automated detection of pillar displacement, estimating muscle contraction.
  • Fig. S9. Significant loss of CNTF secretion in iALS-MN accelerated apoptosis of muscle cells.
  • Fig. S10. Drug application through the iEC barrier.
  • Table S1. Primer sequences for real-time RT-PCR.
  • Table S2. SNP mutation (whole-exome sequencing), ALS pathogenesis related.
  • Table S3. SNP mutation (whole-exome sequencing), ATG family.
  • Table S4. SNP mutation (whole-exome sequencing), autophagy related.
  • Legends for movies S1 to S6

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Other Supplementary Material for this manuscript includes the following:

  • Movie S1 (.mp4 format). Image stacks of muscle fiber bundle stained with α-actinin (green) and DAPI (blue).
  • Movie S2 (.avi format). 3D construction of muscle fiber bundle based on iPSC-derived skeletal muscle cells.
  • Movie S3 (.mp4 format). Representative movie of muscle contraction after stimulation with glutamic acid on day 14.
  • Movie S4 (.mp4 format). Muscle contraction of the ALS motor unit after 1-Hz optical stimulation without drug.
  • Movie S5 (.mp4 format). Muscle contraction of the ALS motor unit after 1-Hz optical stimulation with rapamycin.
  • Movie S6 (.mp4 format). Muscle contraction of the ALS motor unit after 1-Hz optical stimulation with rapamycin and bosutinib.

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