Abbreviations AS, Alport syndrome COL4A1, collagen type IV alpha 1 chain COL4A2, collagen type IV alpha 2 chain Collagen IV is a type of collagen that forms a major component of the basement membrane, which provides support to tissues and a platform for cellular signalling [[1]]. In humans, collagen IV is encoded by six genes, COL4Α1–COL4A6; the α chains encoded by these genes form three heterotrimers, α1α1α2(IV), α3α4α5(IV), and α5α5α6(IV), which then assemble into a sheet-like network [[1-3]].