A novel human iPSC model of COL4A1/A2 small vessel disease unveils a key pathogenic role of matrix metalloproteinases in extracellular matrix abnormalities
M. Al-Thani, M. Goodwin-Trotman, S. Bell…·A. Granata
This paper develops a human iPSC model of COL4A1/A2 small vessel disease, showing that MMP inhibition rescues ECM abnormalities and mural cell defects.