We show that FAZF is a transcriptional repressor that is able to bind to the same DNA target sequences as PLZF. Consistent with a role in FA, BTB/POZ-containing proteins have been implicated in oncogenesis, limb morphogenesis, hematopoiesis, and proliferation. FAZF is homologous to the promyelocytic leukemia zinc finger (PLZF) protein, which has been shown to act as a transcriptional repressor by recruitment of nuclear corepressors (N-CoR, Sin3, and HDAC1 complex). We show that the novel gene, FAZF, encodes a 486 amino acid protein containing a conserved amino terminal BTB/POZ protein interaction domain and three C-terminal Krüppel-like zinc fingers. We have isolated a binding partner for the Fanconi anemia group C protein (FANCC) by yeast two-hybrid screening. At least eight complementation groups (A-H) exist, and although three of the corresponding complementation group genes have been cloned, they lack recognizable motifs, and their functions are unknown. The phenotype includes developmental defects, bone marrow failure, and cell cycle abnormalities. Fanconi anemia (FA) is an autosomal recessive cancer susceptibility syndrome.
