Mixing 'good and bad' annoys neutrophils

摘要

In this issue of Blood, Schurch et al show that signal recognition particle 54 (SRP54) mutations found in patients with congenital neutropenia act in a dominant-negative manner on the functionality of the wild-type SRP54, leading to neutropenia because of impaired splicing of the transcription factor X-box binding protein 1 (XBP1).(1)