To date, DDRGK1 is mostly considered a substrate of UFMylation, which conjugates the target protein with ubiquitin‐fold modifier 1 (UFM1).[14] Although it has recently been shown to play a role in UFMylation‐mediated regulation of erythroid development,[15] spondyloepimetaphyseal dysplasia,[16] and plasma cell development,[17] the mechanism by which DDRGK1 regulates UFMylation and its other functions remain largely unknown. Here, DDRGK1 is linked to spondyloepimetaphyseal dysplasia, matrilin-3 type.