The homeobox DNA-binding domain is a 60 amino acid motif that is conserved among many species and functions to bind DNA via a helix-turn-helix structure, thereby playing a role in transcriptional regulation and the control of gene expression. PRX1 (paired related homeobox 1), also known as PRRX1, PMX1 or PHOX1, is a 245 amino acid protein that contains one OAR domain and one homeobox DNA-binding domain and belongs to the paired homeobox family. Localized to the nucleus, PRX1 functions as a transcriptional co-activator that enhances the DNA-binding activity of serum response factor (SRF), thereby mediating the induction of SRF-dependent gene expression by growth and differentiation factors. Additionally, PRX1 regulates the transcriptional activities of creatine kinase-M (muscle), thereby playing a role in the establishment of mesodermal muscle types. PRX1 exists as two alternatively spliced isoforms, designated PMX1-A and PMX1-B.