Abstract
Collagen IV nephropathies include classical Alport syndrome (AS) with X-linked, autosomal recessive or autosomal dominant inheritance, caused my mutations in COL4A3/A4/A5 genes. Patients heterozygous for COL4A3 or COL4A4 mutations present with microscopic hematuria, due to thin basement membranes (formerly considered a benign condition) and may or may not progress to severe kidney function decline, as an autosomal dominant form of later-onset AS.
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