Intriguingly, a deletion in Col4a5 resulting in disruption of the Gly-X-Y repeats, similar to human p.Pro167Glnfs*36 mutation, was observed in a family of mixed-breed dogs with an inherited nephropathy that exhibits the clinical, immunohistochemical, pathological, and ultrastructural features of human XLAS, and the truncated peptide chain may probably prevent extracellular assembly in type IV collagen networks [28]. Here, COL4A5 is linked to X-linked hydrocephalus with stenosis of the aqueduct of Sylvius.