Fig. (1) Glomerular Proteins in Hereditary Renal Disease. Nephrin, podosin, PLCε1, Myo1E, Arhgap2, GLEPP1 (PTPRO) are podocyte proteins associated with autosomal recessive nephrotic syndrome. TRPC6, INF2, and α-ACTN4 are proteins mutated in autosomal dominant nephrotic syndrome. Mutations in the mitochondrial enzyme co-enzyme Q6 (COQ6) and the α-3 subunit (ITGα3) of the transmembrane integrin are also associated with nephrotic syndrome. Mutations in the glomerular basement membrane (GBM) proteins - collagen IV α 3,4,5 chains and laminin II cause renal disease. Lecithin-cholesterol acyltransferase deficiency leads to lipid deposits in the GBM and renal disease. Laminated apolipoprotein (APOE) deposits are found in the capillary in lipoprotein glomerulopathy.