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Dlx is a family of homeodomain transcription factors which are related to the Drosophila distal-less (Dll) gene [1].

The family has been related to a number of developmental features. The family seems to be well preserved across species[2].

Known members of the family include Dlx1 to Dlx7. They form bigene clusters with each other. There are Dlx1-Dlx2, Dlx5-Dlx6 and Dlx3-Dlx7 clusters in vertebrates. Each of those are linked to a specific Hox-gene cluster. In higher fishes, like zebrafish, there are a couple of additional dlx-genes, dlx5 and dlx8. In zebrafish the orthologous genes to vertebrate Dlx5-Dlx6 are dlx4 and dlx6, which form a bigene cluster in zebrafish. These additional genes are not linked with each other, or any other dlx-gene.

Dlx4, Dlx7, Dlx8 and Dlx9 are the same gene in vertebrates. They're named differently, because every time the same gene was found, the researchers thought they had found a new gene.

Dlx genes are required for the tangential migration of interneurons from the subpallium to the pallium during vertebrate brain development [3]. It has been suggested that Dlx promotes the migration of interneurons by repressing a set of proteins that are normally expressed in terminally differentiated neurons and act to promote the outgrowth of dendrites and axons [4]. Mice lacking Dlx1 exhibit electrophysiological and histological evidence consistent with delayed-onset epilepsy [5].

Dlx2 has been associated with a number of areas including development of the zona limitans intrathalamica and the prethalamus.

Dlx5/6 expression is necessary for normal lower jaw patterning in vertebrates [6].

Dlx7 is expressed in bone marrow[7].


  1. ^ Panganiban G; Rubenstein JL. (October 2002). "Developmental functions of the Distal-less/Dlx homeobox genes.". Development 129 (20): 4371–86. PMID 12223397.  
  2. ^ Stock DW; Ellies DL, Zhao Z, Ekker M, Ruddle FH, Weiss KM. (October 1996). "The evolution of the vertebrate Dlx gene family.". Proc Natl Acad Sci U S A. 93 (20): 10858–63. doi:10.1073/pnas.93.20.10858. PMID 8855272.  
  3. ^ Anderson SA; Eisenstat DD, Shi L, Rubenstein JL. (October 1997). "Interneuron migration from basal forebrain to neocortex: dependence on Dlx genes.". Science. 278 (5337): 474–6. doi:10.1126/science.278.5337.474. PMID 9334308.  
  4. ^ Cobos I; Borello U, Rubenstein JL. (June 2007). "Dlx transcription factors promote migration through repression of axon and dendrite growth.". Neuron. 54 (6): 873–88. doi:10.1016/j.neuron.2007.05.024. PMID 17582329.  
  5. ^ Cobos I; Calcagnotto ME, Vilaythong AJ, Thwin MT, Noebels JL, Baraban SC, Rubenstein JL. (August 2005). "Mice lacking Dlx1 show subtype-specific loss of interneurons, reduced inhibition and epilepsy.". Nat Neurosci. 8 (8): 1059–68. doi:10.1038/nn1499. PMID 16007083.  
  6. ^ Depew MJ; Lufkin T, Rubenstein JL (October 2002). "Specification of jaw subdivisions by Dlx genes". Science. 298 (5592): 381–85.. doi:10.1126/science.1075703. PMID 12193642.  
  7. ^ Takashi Shimamoto,; Shuji Nakamura, Jacques Bollekens, Frank H. Ruddle, and Kenichi Takeshita (April 1997). "Inhibition of DLX-7 homeobox gene causes decreased expression of GATA-1 and c-myc genes and apoptosis". Proc Natl Acad Sci U S A. 94 (7): 3245–9.. doi:10.1073/pnas.94.7.3245. PMID 9096378.  


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