POSTER
Generation of Human iPSC-Derived Duchenne Muscular Dystrophy Skeletal Myocytes Suitable for 3D Functional Studies and Investigating Methods for Dystrophin Restoration
This poster, originally presented by bit.bio at the ISSCR 2024 event in Hamburg, in July 2024, shows how ioSkeletal Myocytes can be used to create functional 3D skeletal muscle microtissues which exhibit twitch and tetanic responses that strengthen over time..
Figure 1: Calcium flux of wild type (WT) ioSkeletal Myocytes under twitch (left) and tetanic (right) stimulation using Fluo-4AM, 1.2s after initial stimulation.
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If you want to:
- Learn more about how iPSC-derived cells were incorporated in the Bi/ond OoC culture system
- Discover what types of phenotypic as well as functional readouts can be made using the MUSbit™ chip
- See an example of how your disease model research can be implemented in an advanced 3D culture platform
Why does it matter?
Using bit.bio's ioSkeletal Myocytes, researchers created functional 3D skeletal muscle microtissues within Bi/ond's MUSbit™ organ-on-chip platform.
These microtissues exhibited twitch and tetanic contraction profiles that strengthened over time. Additionally, the data showed contractions in response to electrical stimulation could be modulated by various compounds. Furthermore, bit.bio introduced deletions of exon 44 (Del Ex44) or exon 52 (Del Ex52) in the Duchenne Muscular Dystrophy (DMD) gene of wild type ioSkeletal Myocytes to model DMD.
With the MUSbit™ microchip, impaired functional activity in the DMD ioSkeletal Myocytes was observed, including reduced twitch, tetanic and sustained contractions in response to electrical stimulation.
.png?width=745&height=468&name=WT%20vs%20DMD%20D14%20Fatigue%20time%20course%20(1).png "WT vs DMD D14 Fatigue time course (1)")
Figure 2: Sustained Contraction readings of three isogenic sets of ioSkeletal myocyte cells; Wild Type (WT) DMD Deletion Exon 44 (DMD Del Ex44) and DMD Deletion Exon 52 (DMD Del Ex52)
The research demonstrated the capability to create a reproducible isogenic system for investigating Duchenne muscular dystrophy. This novel system has the potential to uncover new in vitro functional phenotypes, improve drug screening specificity, and accelerate drug development.
Our groundbreaking microphysiological system establishes a path towards creating more physiologically relevant biomimetic models for advancing drug development and disease modeling