ECD and snowflake vitreoretinal degeneration: Motivated by recent evidence implicating the TGF-β pathway in sporadic and familial SVD [32,33] and by the identification of fibronectin, fibrillin-1 and members of the LTBP family in brain fractions enriched for Notch3 deposits [30], we studied the role of these ECM constituents in CADASIL-related Notch3-ECD aggregation.