The use of spontaneously occurring mouse models has proved to be a valuable tool throughout the years to delineate the signals required for nervous system development. This is especially true in the field of myelin biology, with a large number of different models available. The quaking viable mouse models dysmyelination in the nervous system and links the QUAKING RNA binding proteins to myelination and cell fate decisions. In this Mini‐Review, we highlight the biological functions attributed to this KH‐type RNA binding protein and the recent achievements linking it to human disorders. © 2007 Wiley‐Liss, Inc.