Bioinformatics Database
DLX2: Distal-less homeobox 2
Cellular Process
Cap stage of tooth development
Gene Name
DLX2: Distal-less homeobox 2
Gene ID
1746
Gene Sequence
General Description
Members of the Dlx gene family contain a homeobox that is related to that of Distal-less (Dll), a gene expressed in the head and limbs of the developing fruit fly. The Distal-less (Dlx) family of genes comprises at least 6 different members, DLX1-DLX6. The DLX proteins are postulated to play a role in forebrain and craniofacial development. This gene is located in a tail-to-tail configuration with another member of the gene family on the long arm of chromosome 2.
Alternative titles; symbols
TES1
Chromosome
Chromosome 2
Cytogenetic location
2q31.1
Encoded Protein
Homeobox protein DLX-2
https://www.ncbi.nlm.nih.gov/protein/NP_004396.1/
Function of the protein in oral and tooth development
A bicoid-like homeodomain transcription factor (Pitx2), is known to play roles in tooth development. PITX2 binds to bicoid and bicoid-like elements in the DLX2 promoter and activates this promoter 30-fold in Chinese hamster ovary cells (Espinoza et al., 2002). As Pitx2, Msx2, and Dlx2 are expressed in the dental epithelium, Green et al., (2001) conducted a study to examine the transcriptional activity of PITX2 in concert with Msx2 and the Dlx2 promoter. The results show that h Dlx2 is a target of PITX2 in tooth morphogenesis. While Msx2 can attenuate Dlx2 expression, factors in the tooth epithelium at later stages may interact with PITX2 to attenuate its transcriptional activity. The expression patterns of Pitx2, Msx2, and Dlx2 suggest that Msx2 may regulate Dlx2 to a reduced level in the distal region of the mandible. It is possible that PITX2 plays role to increase Dlx2 expression at the sites of tooth development (Green et al., 2001).
Thomas et al. (1997) identified that a subpopulation of cranial neural crest is specified as odontogenic by Dlx-1 and Dlx-2 genes. Loss of function of Dlx-1 and Dlx-2 results in reprogramming of this population of ectomesenchyme cells into chondrocytes.
Lézot et al, (2000) showed a prominent epithelial involvement of Dlx-2, with stage-specific variations in the cells involved in enamel formation.
Dental and Oral Diseases
Protein Sequence
>NP_004396.1 homeobox protein DLX-2 [Homo sapiens]
MTGVFDSLVADMHSTQIAASSTYHQHQQPPSGGGAGPGGNSSSSSSLHKPQESPTLPVSTATDSSYYTNQ
QHPAGGGGGGGSPYAHMGSYQYQASGLNNVPYSAKSSYDLGYTAAYTSYAPYGTSSSPANNEPEKEDLEP
EIRIVNGKPKKVRKPRTIYSSFQLAALQRRFQKTQYLALPERAELAASLGLTQTQVKIWFQNRRSKFKKM
WKSGEIPSEQHPGASASPPCASPPVSAPASWDFGVPQRMAGGGGPGSGGSGAGSSGSSPSSAASAFLGNY
PWYHQTSGSASHLQATAPLLHPTQTPQPHHHHHHHGGGGAPVSAGTIF
Mutations
Related Literature
Green et al., (2001). https://doi.org/10.3727/000000001783992515
Espinoza et al., (2002): https://doi.org/10.1093/hmg/11.7.743
Thomas, B. L., Tucker, A. S., Qui, M., Ferguson, C. A., Hardcastle, Z., Rubenstein, J. L., & Sharpe, P. T. (1997). Role of Dlx-1 and Dlx-2 genes in patterning of the murine dentition. Development (Cambridge, England), 124(23), 4811–4818.
Lézot et al. (2000). https://doi.org/10.1359/jbmr.2000.15.3.430
