Overall, major strengths of this work are as follows: (1) we generated a new hiPSC-derived disease model for SVD, which replicates phenotypic changes observed in patients and Col4a1 animal model, including ECM abnormalities, and (2) this disease-relevant model can be used as new tool for the analysis of signaling pathways to identify therapeutic targets, such as specific MMP, and (3) to screen and test for potential drugs against SVD. This evidence concerns the gene COL4A1 and snowflake vitreoretinal degeneration.