BPoD has moved!

BPoD has recently changed our domain name - we can now be found at bpod.org.uk

Please update your bookmarks!

Now in our 13th year of bringing you beautiful imagery from biomedical science every day

Search the archive of over 4000 images

Model Muscle

3D skeletal muscle fibre bundle model for investigating muscle disease and treatments

22 May 2021

Model Muscle

Infantile-onset Pompe disease (IOPD) is a rare but life-threatening disease. It causes skeletal and heart muscle weakness due to a deficiency in an enzyme called acid alpha-glucosidase (GAA). Current treatment with healthy GAA doesn’t provide a complete cure. Researchers now investigate why using the first-ever 3D human skeletal muscle model in a dish. Bundles of human muscle fibres, myobundles (pictured), were grown from muscle cells collected from healthy (left) and IOPD patients (right). IOPD myobundles replicated patient symptoms — reduced GAA enzyme activity, elevated glycogen levels and poor muscle function when under metabolic stress. Treating these myobundles with human GAA, increased GAA activity and reduced glycogen levels as shown by measuring specific protein levels. But this didn’t improve muscle function under stress, as demonstrated by measuring forces generated when myobundles contracted. This model, therefore, presents an effective way to gain new insights into IOPD and potential treatments.

Written by Lux Fatimathas

Search The Archive

Submit An Image

Follow on Tumblr

Follow on Instagram

What is BPoD?

BPoD stands for Biomedical Picture of the Day. Managed by the MRC Laboratory of Medical Sciences until Jul 2023, it is now run independently by a dedicated team of scientists and writers. The website aims to engage everyone, young and old, in the wonders of biology, and its influence on medicine. The ever-growing archive of more than 4000 research images documents over a decade of progress. Explore the collection and see what you discover. Images are kindly provided for inclusion on this website through the generosity of scientists across the globe.

Read More

BPoD is also available in Catalan at www.bpod.cat with translations by the University of Valencia.