The ZNF384 (zinc-finger protein 384, a.k.a. CIZ) gene encodes a transcription factor involved in the regulation of matrix metalloproteinases.

Rearrangements of the ZNF384 gene are recurrent in acute leukemia and are most frequently found in precursor B-cell acute lymphoblastic leukemia (BCP-ALL) in children and young adults with an incidence of about 3-4%. ZNF384-related fusion genes with multiple fusion partners have been found to define a distinct subgroup of pediatric BCP-ALL with a characteristic immunophenotype. Known translocation partners are TCF3 (19p13.3), EWSR1 (22q12.2), TAF15 (17q12), EP300 (22q13.2), ARID1B (6q25.3), CREBBP (16p13.3), and BMP2K (4q21.21) with TCF3 being the most prevalent. The breakpoints are located within the ZNF384 gene. However, the balanced translocations are resulting in fusion genes including the complete protein coding information. Since ZNF384-related fusion genes are difficult to detect by common G-banding, investigation by FISH may be of diagnostic and prognostic relevance.