Proteomics-based assays have substantially improved diagnostic accuracy in systemic amyloidosis and have led to the discovery of novel amyloidogenic proteins. The observation that human apolipoproteins are increasingly represented is intriguing and highlights the complex, and partially known, mutual effects of the misfolded proteins and lipids. Dasari et al. report on the first series of patients affected by the recently described apolipoprotein A-IV amyloidosis, detailing its peculiar clinical and pathologic findings and diagnostic pitfalls.
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