The protein encoded by this gene is a homeodomain-containing transcription factor that appears to function in the regulation of organogenesis. This gene is located downstream of the dystrophia myotonica-protein kinase gene. Mutations in this gene are a cause of branchiootorenal syndrome type 2.SIX5 (SIX Homeobox 5) is a Protein Coding gene. Diseases associated with SIX5 include Branchiootorenal Syndrome 2 and Branchiootorenal Syndrome. GO annotations related to this gene include sequence-specific DNA binding. An important paralog of this gene is SIX4.