Gene Symbol: Tlx2
Description: T cell leukemia, homeobox 2
Alias: Enx, Hox11L.1, Hox11l1, NCX, Ncx1, Tlx1l1, Tlx1l2, T-cell leukemia homeobox protein 2, PMUR10F, enteric neuron homeobox protein, homeo box 11-like 1, homeobox TLX-2, homeobox protein Hox-11L1
Species: mouse
Products:     Tlx2

Top Publications

  1. Shirasawa S, Yunker A, Roth K, Brown G, Horning S, Korsmeyer S. Enx (Hox11L1)-deficient mice develop myenteric neuronal hyperplasia and megacolon. Nat Med. 1997;3:646-50 pubmed
    The isolated homeobox gene Enx (Hox11L1) is expressed in enteric neurons innervating distal ileum, and proximal and distal colon. Enx-deficient mice develop megacolon with massive distension of the proximal colon...
  2. Oki T, Yamazaki K, Kuromitsu J, Okada M, Tanaka I. cDNA cloning and mapping of a novel subtype of glutamine:fructose-6-phosphate amidotransferase (GFAT2) in human and mouse. Genomics. 1999;57:227-34 pubmed
    ..GFAT2 can provide insights into understanding the roles of the hexosamine pathway in various tissues, particularly with the development of glucose toxicity and diabetes complications. ..
  3. Parisi M, Lipman N, Clarke C, Taylor B, Kapur R. Evaluation of Hox11L1 in the fmc/fmc rat model of chronic intestinal pseudo-obstruction. J Pediatr Surg. 2005;40:1760-5 pubmed
    ..fmc), is responsible for an autosomal recessive phenotype similar to intestinal pseudo-obstruction observed in Hox11L1-/- mice...
  4. Roberts C, Sonder A, Lumsden A, Korsmeyer S. Development expression of Hox11 and specification of splenic cell fate. Am J Pathol. 1995;146:1089-101 pubmed
    ..1 and Hox1L1.2, were not expressed in a redundant pattern. Neither Hox11L1 nor Hox11L2 was expressed in the branchial arches or their motor nuclei within wild-type or Hox11-/- mice...
  5. Aoki T, Jusuf A, Iitsuka Y, Isono K, Tokuhisa T, Hatano M. Ncx (Enx, Hox11L.1) is required for neuronal cell death in enteric ganglia of mice. J Pediatr Surg. 2007;42:1081-8 pubmed
    b>Ncx (Enx, Hox11L.1)-deficient (Ncx-/-) mice develop mega-ileo-ceco-colon with a larger number of neuronal cells in the enteric ganglia...
  6. Qian Y, Fritzsch B, Shirasawa S, Chen C, Choi Y, Ma Q. Formation of brainstem (nor)adrenergic centers and first-order relay visceral sensory neurons is dependent on homeodomain protein Rnx/Tlx3. Genes Dev. 2001;15:2533-45 pubmed
  7. Kennedy M, Gonzalez Sarmiento R, Kees U, Lampert F, Dear N, Boehm T, et al. HOX11, a homeobox-containing T-cell oncogene on human chromosome 10q24. Proc Natl Acad Sci U S A. 1991;88:8900-4 pubmed
    ..The coding capacity of this activated gene, designated HOX11, is undisturbed in a T-cell line carrying the translocation t(7;10)(q35;q24). Therefore, the HOX11 homeobox gene seems to be involved in T-cell tumorigenesis. ..
  8. Kapur R, Clarke C, Doggett B, Taylor B, Baldessari A, Parisi M, et al. Hox11L1 expression by precursors of enteric smooth muscle: an alternative explanation for megacecum in HOX11L1-/- mice. Pediatr Dev Pathol. 2005;8:148-61 pubmed
    Previous studies have focused on expression of Hox11L1 in enteric neurons as the explanation for intestinal and urinary bladder dysmotility observed in mice that do not have the transcription factor...
  9. Raju K, Tang S, Dube I, Kamel Reid S, Bryce D, Breitman M. Characterization and developmental expression of Tlx-1, the murine homolog of HOX11. Mech Dev. 1993;44:51-64 pubmed

Scientific Experts

More Information


  1. Ciciotte S, Tsai F, Peters L. Gata2 maps to mouse chromosome 6. Mamm Genome. 1997;8:69-70 pubmed
  2. Wen X, Tang S, Breitman M. Genetic mapping of two mouse homeobox genes Tlx-1 and Tlx-2 to murine chromosomes 19 and 6. Genomics. 1994;24:388-90 pubmed
    ..The proposed gene orders and genetic distances for Tlx-1 and Tlx-2 are centromere 19-Lpc-1-(25.53 cM)-Pltr-4-(5.32 cM)-Tlx-1- (3.19 cM)-Ins-1-(7.45 cM)-Xmv-18, and centromere 6-Tcrb-(12.90 cM)-Mltr-3-(10.75 cM)-Tlx-2-(18.42 cM)-Xmv-6. ..
  3. Bersohn M, Carmack C, Philipson K. Ischemic tolerance of homozygous transgenic mouse hearts overexpressing the sodium-calcium exchanger. Ann N Y Acad Sci. 2002;976:483-6 pubmed
  4. Xiao C, Shim J, Kl├╝ppel M, Zhang S, Dong C, Flavell R, et al. Ecsit is required for Bmp signaling and mesoderm formation during mouse embryogenesis. Genes Dev. 2003;17:2933-49 pubmed
    ..Therefore, these results show that Ecsit functions as an essential component in two important signal transduction pathways and establishes a novel role for Ecsit as a cofactor for Smad proteins in the Bmp signaling pathway. ..
  5. Tang S, Suen T, McInnes R, Buchwald M. Association of the TLX-2 homeodomain and 14-3-3eta signaling proteins. J Biol Chem. 1998;273:25356-63 pubmed
    ..These findings define 14-3-3eta as a functional modulator of the TLX-2 homeodomain transcription factor and suggest that the in vivo function of TLX-2 in neural differentiation is likely regulated by signaling mediated by 14-3-3eta. ..
  6. Hatano M, Aoki T, Dezawa M, Yusa S, Iitsuka Y, Koseki H, et al. A novel pathogenesis of megacolon in Ncx/Hox11L.1 deficient mice. J Clin Invest. 1997;100:795-801 pubmed
    The Ncx/Hox11L.1 gene, a member of the Hox11 homeobox gene family, is mainly expressed in neural crest-derived tissues. To elucidate the role of Ncx/Hox11L...
  7. Heyer B, Warsowe J, Solter D, Knowles B, Ackerman S. New member of the Snf1/AMPK kinase family, Melk, is expressed in the mouse egg and preimplantation embryo. Mol Reprod Dev. 1997;47:148-56 pubmed
    ..This gene product may play a role in the signal transduction events in the egg and early embryo. ..
  8. Howe D, Clarke C, Yan H, Willis B, Schneider D, McKnight G, et al. Inhibition of protein kinase A in murine enteric neurons causes lethal intestinal pseudo-obstruction. J Neurobiol. 2006;66:256-72 pubmed
    ..subunit (RIalphaB) of PKA was activated in enteric neurons by either a Proteolipid protein-Cre transgene or a Hox11L1-Cre "knock-in" allele...
  9. Parisi M, Baldessari A, Iida M, Clarke C, Doggett B, Shirasawa S, et al. Genetic background modifies intestinal pseudo-obstruction and the expression of a reporter gene in Hox11L1-/- mice. Gastroenterology. 2003;125:1428-40 pubmed
    The transcription factor Hox11L1 is expressed by enteric neurons. Two groups mutated murine Hox11L1, and reported lethal intestinal pseudo-obstruction and colonic hyperganglionosis in many, but not all, homozygous null mutants...
  10. Iitsuka Y, Shimizu H, Kang M, Sasagawa K, Sekiya S, Tokuhisa T, et al. An enhancer element for expression of the Ncx (Enx, Hox11L1) gene in neural crest-derived cells. J Biol Chem. 1999;274:24401-7 pubmed
    The murine Ncx (Enx, Hox11L1) gene is specifically expressed in a neuronal subset of neural crest-derived tissues...
  11. Ji W, Chen F, Do T, Do A, Roe B, Meisler M. DQX1, an RNA-dependent ATPase homolog with a novel DEAQ box: expression pattern and genomic sequence comparison of the human and mouse genes. Mamm Genome. 2001;12:456-61 pubmed
    ..The 3.2-kb Dqx1 transcript has highest expression in muscle and liver. DQX1 is located between AUP1 and HOX11L1 in a gene-dense region of human Chromosome (Chr) 2p13 and mouse Chr 6...
  12. Dear T, Sanchez Garcia I, Rabbitts T. The HOX11 gene encodes a DNA-binding nuclear transcription factor belonging to a distinct family of homeobox genes. Proc Natl Acad Sci U S A. 1993;90:4431-5 pubmed
    ..These all contain a threonine residue in place of the more common isoleucine or valine in helix 3 of the homeodomain. HOX11 therefore appears to belong to a family of DNA-binding transactivators of transcription. ..
  13. Puliti A, Cinti R, Betsos N, Romeo G, Ceccherini I. HOX11L1, a gene involved in peripheral nervous system development, maps to human chromosome 2p13.1-->p12 and mouse chromosome 6C3-D1. Cytogenet Cell Genet. 1999;84:115-7 pubmed
    b>HOX11L1 is a homeobox gene involved in peripheral nervous system development as confirmed by knockout mice exhibiting megacolon with enteric ganglia, a phenotype associated in human with Intestinal Neuronal Dysplasia (IND)...
  14. Kintner D, Luo J, Gerdts J, Ballard A, Shull G, Sun D. Role of Na+-K+-Cl- cotransport and Na+/Ca2+ exchange in mitochondrial dysfunction in astrocytes following in vitro ischemia. Am J Physiol Cell Physiol. 2007;292:C1113-22 pubmed
    Na(+)-K(+)-Cl(-) cotransporter isoform 1 (NKCC1) and reverse mode operation of the Na(+)/Ca(2+) exchanger (NCX) contribute to intracellular Na(+) and Ca(2+) overload in astrocytes following oxygen-glucose deprivation (OGD) and ..
  15. Bernard O, Busson LeConiat M, Ballerini P, Mauchauffe M, Della Valle V, Monni R, et al. A new recurrent and specific cryptic translocation, t(5;14)(q35;q32), is associated with expression of the Hox11L2 gene in T acute lymphoblastic leukemia. Leukemia. 2001;15:1495-504 pubmed
    ..These data establish the t(5;14)(q35;q32) as a major abnormality, and Hox11 family member activation as an important pathway in T ALL leukemogenesis. ..
  16. Nunes M, Blanc I, Maes J, Fellous M, Robert B, McElreavey K. NSPc1, a novel mammalian Polycomb gene, is expressed in neural crest-derived structures of the peripheral nervous system. Mech Dev. 2001;102:219-22 pubmed
    ..At 11.5 dpc, transcripts are present in a subset of neural crest cell derivatives of the peripheral nervous system, and in the neural tube. NSPc1 expression is ubiquitous in adult tissue. ..
  17. Tang S, Hoodless P, Lu Z, Breitman M, McInnes R, Wrana J, et al. The Tlx-2 homeobox gene is a downstream target of BMP signalling and is required for mouse mesoderm development. Development. 1998;125:1877-87 pubmed
    ..Similar to BMP4 and ALK3 mutants, the mutant embryos display severe defects in primitive streak and mesoderm formation. These experiments thus define a BMP/Tlx-2 signalling pathway that is required during early mammalian gastrulation. ..
  18. Neco P, Rose B, Huynh N, Zhang R, Bridge J, Philipson K, et al. Sodium-calcium exchange is essential for effective triggering of calcium release in mouse heart. Biophys J. 2010;99:755-64 pubmed publisher
    ..Although Na+-Ca2+ exchange (NCX) is essential for Ca2+ extrusion, its participation in the trigger process of excitation-contraction coupling is ..