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. HMX2 (H6 family homeobox 2), also known as H6L or Nkx5-2, is a 273 amino acid nuclear protein that belongs to the HMX homeobox family and contains one homeobox DNA-binding domain. HMX2 functions as a transcription factor that assists in specification of neuronal cell types and is essential for proper development of hypothalamus and inner ear. Hemizygous deletions of the gene encoding HMX2 are thought to lead to vestibular dysfunction, inner ear malformations and congenital sensorineural hearing loss.