3752-511_3955 + 576 del, or missense variant p. (Gly1155Asp) demonstrated the typical ultrastructural basket-weave change in the GBM or a thin GBM and an absence of type IV collagen α5 chain in the GBM and only normal immunostaining of Bowman's capsule (typical immunofluorescence staining pattern for autosomal recessive Alport syndrome) (28), suggesting these COL4A3 variants affect type IV collagen α3 chain production. Here, COL4A3 is linked to autosomal recessive Alport syndrome.