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- Published: April 17, 2020
- By: Tiffany Caza, MD
The above painting depicts electron microscopy of a case of Alport syndrome. Alport syndrome is a type of hereditary nephritis due to mutations in the alpha 3, alpha 4, or alpha 5 chains of collagen type IV. As a result, the typical alpha 3-4-5 meshwork of type IV collagen in the GBM is replaced by an alpha 1-2 meshwork, which has less tensile strength and has susceptibility to proteolysis by matrix metalloproteinases (Zeisberg et al, 2006). Patients with Alport syndrome present with hematuria and progressive renal failure, and males are disproportionately affected. Deafness and visual problems can also occur. Kidney...