Zinc finger HIT-type containing 6 (ZNHIT6)

Zinc finger HIT-type containing 6 (ZNHIT6) is a protein-coding gene located on human chromosome 1 that encodes an essential assembly factor for box C/D small nucleolar ribonucleoproteins (snoRNPs)[1][5]. ZNHIT6, also known as BCD1 and NY-BR-75 among other names, contains a zinc finger HIT-type domain (ZHD) and functions mainly as an RNA-binding protein critical for the maturation, accumulation, and nucleolar transport of box C/D snoRNAs, which direct the chemical modification (2′-O-methylation) and processing of rRNAs during ribosome assembly[1][2][3][6]. ZNHIT6 acts during the early steps of snoRNP assembly and is necessary for maintaining steady-state levels of box C/D snoRNAs, thus enabling efficient ribosome biogenesis and, ultimately, protein synthesis in eukaryotic cells[2][3]. Mutations or fusion events (e.g., MOV10-ZNHIT6) have been observed in some cancer types, implicating ZNHIT6 in tumor biology, although the protein itself is predominantly a housekeeping factor without direct evidence for therapeutic targeting. No drugs are currently known to directly target ZNHIT6, and its essential cellular functions raise concerns about tolerability of direct inhibition.

Other names

BCD1Box C/D snoRNA protein 1C1orf181FLJ20729NY-BR-75Serologically defined breast cancer antigen NY-BR-75Zinc finger HIT domain-containing protein 6box C/D snoRNA essential 1 homolog (S. cerevisiae)box C/D snoRNA protein 1box C/D snoRNA essential 1 homologzinc finger, HIT type 6