Zmym2, also known as ZNF198, is a zinc finger transcriptional regulator and a component of transcriptional corepressor complexes. It is involved in multiple crucial biological processes. Zmym2 plays a role in the silencing of developmentally regulated endogenous retrovirus elements, and is associated with pathways related to DNA methylation, chromatin regulation, and cell identity maintenance [2,3,4].

Loss-of-function studies have been pivotal in understanding Zmym2's role. Heterozygous Zmym2 -deficient mice recapitulate features of congenital anomalies of the kidney and urinary tract (CAKUT) with high penetrance, indicating Zmym2's importance in renal development [1]. Zmym2 -/-mice show embryonic lethality by E10.5, with defects in DNA methylation and silencing of germline gene promoters and active LINE element subclasses [3]. In addition, zygotic depletion of Zmym2 compromises the totipotency -to -pluripotency transition during early development [2].

In conclusion, Zmym2 is essential for processes like DNA methylation patterning in early embryonic development, the totipotency -to -pluripotency transition, and renal development. Mouse models with Zmym2 knockout or knockdown have been instrumental in revealing its functions, especially in relation to CAKUT, providing insights into the pathogenesis of this disorder and potentially guiding new therapeutic strategies [1,2,3].