This clinical renal presentation contrasts those described for Leu75Pro apoA1 amyloidosis20 and A-alpha chain amyloidosis21, which typically manifests as a chronic tubulo-interstitial nephritis with amyloid deposits restricted to the inner medulla, or as proteinuria/nephrotic syndrome because of massive glomerular amyloid deposition, respectively, ApoC-III should be added to the list of protein variants associated with hereditary renal amyloidosis22, along with apoA-I (ref. 10, apoAII (ref. 11), lysozyme23, fibrinogen Aα-chain21 and gelsolin24. Here, APOC3 is linked to nephrotic syndrome.