Comparison of mesenchymal stromal cell types for cell therapy of UCMD

Type 6 collagen (COL6) is produced by mesenchymal stromal cells (MSC) in skeletal muscle, and its deficiency is the cause of Ulrich congenital muscular dystrophy (UCMD). To test whether COL6 supplementation has a therapeutic effect for UCMD, we conducted in vivo and in vitro experiments using healthy human skeletal muscle tissue-derived primary MSCs, healthy induced pluripotent stem cell (iPSC)-derived MSCs (iMSCs) and COL6-deficient iMSCs (COL6KO-iMSCs). We also compared the mRNA expression profiles of both iMSC types, iPSC types, healthy human skeletal muscle tissue-derived primary MSCs and muscle stem cells.