Trichorhinophalangeal Syndrome Protein I (TRPS1)

TRPS1 encodes a GATA-like transcription factor that represses GATA-regulated genes and binds to a dynein light chain protein. Binding of the encoded protein to the dynein light chain protein affects binding to GATA consensus sequences and suppresses its transcriptional activity. Defects in this gene are a cause of tricho-rhino-phalangeal syndrome (TRPS) types I-III.Region A covers 89 amino acids (635-723), spanning 3 potential C2H2 zinc finger structures, and region B covers the 100 most C-terminal amino acids (1182-1281) containing the IKAROS-like motif. DNCL1 colocalized with TRPS1 in dot-like structures in the cell nucleus. An electrophoretic mobility shift assay showed that the interaction of DNCL1 and TRPS1 lowered the binding of TRPS1 to the GATA consensus sequence.