Abstract
Firstly described in 1927, Alport syndrome is a progressive hereditary nephropathy. Untreated, it leads to end-stage renal failure and is associated with the symptoms of hematuria and proteinuria. The aim of this work was the identification of non-invasive biomarkers in knockout mice urine under nephroprotective therapy with the ACE inhibitor ramipril, allowing clinically relevant predictions in therapy with ACE inhibitors in patients with Alport syndrome. Using Proteomics- and Westernblot-analysis, the urine from SVJ 129 COL4A3 knockout mice at 6-weeks- and 7.5-weeks-stages were compared – each with and without treatment using ACE inhibitors. Three candidate markers were identified. Albumin, Antithrombin III and Haptoglobin may suit for a mouse model of Alport syndrome. They may serve as a reference of the three types of markers: 1. disease progression, 2. treatment success and 3. treatment history.
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