It activates pro-inflammatory pathways via interaction with pattern recognition receptors such as toll-like receptor 4 (TLR4) and RAGE, leading to nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) activation and subsequent release of cytokines including interleukin-6 (IL-6), which has been directly implicated in AAA pathogenesis. This evidence concerns the gene AGER and triple-A syndrome.