The DNA-associated protein encoded by this gene is a member of the paired family of homeobox proteins localized to the nucleus. The protein functions as a transcription co-activator, enhancing the DNA-binding activity of serum response factor, a protein required for the induction of genes by growth and differentiation factors. The protein regulates muscle creatine kinase, indicating a role in the establishment of diverse mesodermal muscle types. Alternative splicing yields two isoforms that differ in abundance and expression patterns.PRRX1 (Paired Related Homeobox 1) is a Protein Coding gene. Diseases associated with PRRX1 include Agnathia-Otocephaly Complex and Dysgnathia Complex. GO annotations related to this gene include sequence-specific DNA binding and HMG box domain binding. An important paralog of this gene is PRRX2.