LZTR1, leucine-zipper-like transcriptional regulator 1, is a member of the BTBkelch superfamily. LZTR1 contains 2 BTB (POZ) domains and 6 Kelch repeats. The BTB (broad-Complex, Tramtrack and Bric a brac) domain, also known as the POZ (POxvirus and Zinc finger) domain, is an N-terminal homodimerization domain that contains multiple copies of kelch repeats and/or C2 H2-type zinc fingers. Proteins that contain BTB domains are thought to be involved in transcriptional regulation via control of chromatin structure and function. LZTR1 is believed to function as a transcriptional regulator during embryogenesis. LZTR1 is expressed in fetal brain, heart, kidney, liver and lung and is found exclusively on the cytoplasmic surface of the Golgi network. LZTR1 likely contributes to the etiology of velocardiofacial/DiGeorge syndrome, as the LZTR1 gene lies within a chromosomal deletion region associated with the disease.