Transcriptional activator that directly regulates early activation of the myogenic determination gene MYF5 by binding in a sequence-specific manner to the early epaxial enhancer element of it. Involved in somitogenesis during embryogenesis and somite development and differentiation into sclerotome and dermomyotome. Required for the initiation and/or maintenance of proper organization of the sclerotome, dermomyotome and myotome. Is not required for sex determination and/or differentiation in embryonic development. Also not involved in symmetric somite formation and hence does not regulate the laterality pathway that controls left-right asymmetric organ positioning. Homodimer. Expressed in testis. Expressed in presomitic mesoderm and developing somites at 8.5 dpc, accumulating in the epaxial domain at 9.5 dpc in the immature caudal somites. At 10.5 dpc expressed in the dermomyotome of somites. By 11.5 dpc, only detectable in caudal somites. In mature somites, expression is confined to the dermomyotome of the somite where Pax3 is also expressed and in the epaxial domain of the most immature caudal somites. At this stage, the most mature anterior somites are beginning to lose the expression at the epaxial and hypaxial extremities of the dermomyotome. At 14.5 dpc expressed in testis, heart and brain. Expression is also detected in proximal forelimb buds and branchial arches of the developing embryo. Transcriptionally up-regulated by PAX3 within the dermomyotome. Mice heterozygous for Dmrt2 mutation exhibit no visible phenotype. Homozygotes are perinatally lethal due to signs of respiratory distress. They have rib, sternal, vertebral and skull base malformations. Expression of myogenic growth factors, such as Pax3 and Myog, the growth factor Pdgfa, cadherin Cdh2, and the filament protein Des is abnormal. Mutants lack most or all of the epithelial organization seen in wild-type somites, sclerotome and dermomyotome. Mutants have disrupted production of matrix components including laminin-1 in the dermomyotome. Arrangement of the myogenic cells of the inter-limb somites is abnormal. Myf5 activation in the epaxial domain is retarded thus having consequences in the myogenesis by delaying expression of Myog. Mutants do not have left-right defects regarding internal organs positioning. Dmrt2 and Pax3 double null mutant embryos exhibit suspended development and Myog expression is markedly decreased and its expression pattern dramatically distorted. Belongs to the DMRT family. Truncated N-terminus.