Zinc finger and BTB domain-containing protein 18 (ZBTB18)

ZBTB18 (Zinc finger and BTB domain-containing protein 18) is a C2H2-type zinc finger transcription factor that acts as a transcriptional repressor essential for the proper timing of neural precursor differentiation and brain development, as well as myogenesis[1][2][3][4]. It recognizes specific DNA motifs and recruits chromatin remodeling complexes to target genes, directly suppressing proneurogenic factors like Pax6, Ngn2, and NeuroD1, ensuring exit from the cell cycle and maturation of neuronal progenitors. Pathogenic variants, particularly within its zinc finger domain, impair DNA binding and disrupt neural circuit formation, contributing to intellectual disability and other neurodevelopmental disorders. In cancer, ZBTB18 functions as a tumor suppressor—often silenced by promoter methylation in gliomas—where its functional loss is associated with enhanced proliferation and therapy resistance. ZBTB18 also has roles in chromatin organization and may participate in immune cell differentiation[1][2][3][4].

Other names

RP58ZNF238TAZ-1C2H2-171MRD2258 kDa repressor proteintranscriptional repressor RP58translin-associated zinc finger protein 1zinc finger protein 238DEL1Q42Q44DEL1Q43Q44C1DELq42q44C1DELq43q44