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In males, testicular AApo AI deposition can occur and hypogonadism may be the first sign of disease [65]. Kidney disease in AApo AI often manifests as slowly progressive renal failure with minimal proteinuria; however, nephrotic syndrome has been reported. Renal amyloid deposits have mainly been reported within the medullary interstitium with sparing of the renal cortex [66]. Two cases of AApo AI with more extensive glomerular and vascular involvement have also been described [30, 67]. Amyloidogenic apolipoprotein AII was first reported in 2001, and all of the reported mutations have occurred at the stop codon resulting in a 21 amino acid extension of the wild-type protein [68, 69]. Clinically, kidney disease dominates and patients typically present with progressive [http://www.selleckchem.com/products/Imatinib-Mesylate.html check details] renal failure and proteinuria. Histologically, AApo AII amyloid deposits are most frequently identified within the glomerulus and small vessels. Extra-renal amyloid deposits have been identified in autopsy cases, primarily within vessel walls [53, 68]. A case of apolipoprotein AIV amyloidosis (AApo AIV) has recently been described with predominantly medullary [http://en.wikipedia.org/wiki/MERTK UNC2881] amyloid deposition [70]. Amyloidosis due to variant apolipoprotein AI, AII or AIV is very rare and accounted for [http://www.selleckchem.com/products/AP24534.html selleck chemicals llc] Transthyretin is predominantly synthesized in the liver and at least 100 different mutations leading to amyloidosis have been described in the TTR gene with up to 15 nephropathic variants [72]. The TTR Val30Met variant is the most common and has a worldwide distribution. Wild-type transthyretin also has the potential to form amyloid fibrils and is implicated in systemic senile amyloidosis. The age of onset can be quite variable, and a family history may be difficult to uncover due to late-onset disease [9, 72]. Kidney involvement is often heralded by microalbuminuria, and in a study, 50% of patients with microalbuminuria progressed to overt nephropathy and 32% of those showed progression to ESRD [73].