Zinc finger transcription factor Trps1 (TRPS1)

Zinc finger transcription factor Trps1 (TRPS1) is an atypical member of the GATA-type transcription factor family, distinguished by its single GATA-type zinc finger and additional Ikaros-type zinc finger domains at the C-terminus[2][4][5]. Unlike typical GATA proteins that function as transcriptional activators, TRPS1 acts as a sequence-specific transcriptional repressor, primarily repressing GATA-regulated genes by binding to consensus GATA sites on DNA and interacting with corepressor complexes such as CHD4/NuRD[1][2][5]. Mutations in TRPS1 cause tricho-rhino-phalangeal syndromes (TRPS types I–III), characterized by craniofacial, skeletal, and hair anomalies[3][4]. TRPS1 is also implicated in the pathogenesis of various cancers, including breast, colon, and osteosarcoma, where it can regulate genes related to cancer progression[1][5]. The repressive activity of TRPS1 is essential for proper development, and both loss and dominant-negative mutations lead to significant developmental disorders[2][3][4]. Currently, there are no known drugs that specifically target TRPS1 directly, nor defined mechanisms of action for therapeutic intervention, but its mutation status is used as a biomarker in clinical genetics[3][5][6].

Other names

Tricho-rhino-phalangeal syndrome type I proteinTranscriptional repressor GATA binding 1LGCRGC79Zinc finger protein GC79