Update: Development of advanced human preclinical models for FSHD modeling

Posted by Friends of FSH Research on Jul 7, 2026

Report by Dr. Stefano Testa
See also Development of advanced human preclinical models for FSHD modeling

The aim of this project is to develop advanced human-based skeletal muscle models capable of faithfully reproducing the main features of FSHD and other muscular diseases. These models could become an e ective alternative to animal testing, allowing faster and more accurate screening of new therapies.

During the first phase of the project, we focused on the development of innovative strategies to model skeletal muscle tissue. We developed an advanced 2D culture system based on topographic surfaces able to guide the alignment of human myoblasts. In parallel, we created an innovative muscle-on-a-chip platform capable of rapidly generating small and well-organized 3D mini-muscles.

In the last six months, we concentrated on improving these models through electrical stimulation. We designed and validated a Pulsed Electrical Stimulation (PES) device able to stimulate both 2D and 3D skeletal muscle models, also enabling functional analyses. Using this technology, we observed that electrically stimulated muscle cells showed improved maturation and became progressively responsive and synchronized to the electrical input.

Another important activity of the project focused on the implementation of a previously developed 3D bioprinting system tailored for skeletal muscle modeling. Thanks to a newly formuled bioink we succeded in improving both the stability and reproducibility of the printing process.

In the final phase of the project, we plan to compare healthy and FSHD skeletal muscle models generated using both the 2D and 3D culture systems. We will also evaluate the effect of electrical stimulation on muscle fiber maturation and on the reproduction of the pathological phenotype.