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, K. Patel, L. K. Fleming, C. Vilain, M. Abramowicz, S. M. Allan, T. Wang, Z. Cader, K. Horsburgh, T. Van Agtmael, S. Sinha, H. S. Markus, 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.
bioRxiv · Feb 23, 2023 · PreprintCell Biology