A human immune/muscle xenograft model of FSHD muscle pathology.
Katelyn DamanJing YanLisa M BurzenskiJamie KadyLeonard D ShultzMichael A BrehmCharles P EmersonPublished in: bioRxiv : the preprint server for biology (2023)
The NSG-SGM3-W41 mouse provides an experimental model to investigate the role of innate immunity and complement in FSHD muscle pathology and to develop FSHD therapeutics targeting DUX4 and the innate immunity inflammatory responses.