The physiologic function of NBAS is not yet understood. In yeast, sec39 is involved in retrograde transport between ER and Golgi.9 Aoki et al.9 provided evidence for a similar function of NBAS in humans where it is interacting with p31. Together with other SNARE proteins, they are forming the syntaxin 18 complex implicated in ER membrane fusion. Concomitant with reduced NBAS levels, p31 levels were also decreased, providing additional evidence that both proteins are subunits of the same SNARE complex. Similar findings were observed after targeted knockdown in HeLa cells.9 The exact mechanism of ALF due to NBAS deficiency remains unclear. We reason that a catabolic state with high energy demand during febrile infections or the raised temperature itself might be the starting points of the derailment, speculatively via a thermal susceptibility of the syntaxin 18 complex.