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Gene Review

DCX  -  doublecortin

Homo sapiens

Synonyms: DBCN, DC, Doublin, LISX, Lis-X, ...
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Disease relevance of DCX


Psychiatry related information on DCX

  • DCX mutations cause mental retardation in male subjects with lissencephalypachygyria and in female subjects with subcortical band heterotopia (SBH) [5].
  • Periods rich in slow-wave sleep (SWS) not only facilitate the consolidation of declarative memories, but in humans, SWS is also accompanied by a pronounced endogenous transcortical DC potential shift of negative polarity over frontocortical areas [6].

High impact information on DCX

  • The protein encoded by this gene is 2,156 amino acids and its function is currently unknown, although the amino terminus has similarity to that of the doublecortin protein, whose gene (DCX) has been implicated in lissencephaly in humans [7].
  • A novel lysosome-associated membrane glycoprotein, DC-LAMP, induced upon DC maturation, is transiently expressed in MHC class II compartment [8].
  • This suggests that DC-LAMP might change the lysosome function after the transfer of peptide-MHC class II molecules to the surface of DC [8].
  • Functionally, preincubation of DCs or resting T cells with blocking neuropilin-1 antibodies inhibited DC-induced proliferation of resting T cells [9].
  • Several studies using in utero electroporation and RNAi have revealed that transition out of the multipolar stage depends on the function of filamin A, LIS1 and DCX [10].

Chemical compound and disease context of DCX


Biological context of DCX


Anatomical context of DCX

  • In functional assays, the N-terminal DCX domain of doublecortin binds only to assembled microtubules, whereas the C-terminal domain binds to both microtubules and unpolymerized tubulin [18].
  • To study the functional consequences of DCX mutations, we overexpressed seven of the reported mutations in COS7 cells and examined their effect on the microtubule cytoskeleton [14].
  • The neuronal migration protein doublecortin (DCX) that associates with microtubules through a tandem DCX repeat, is required for the development of the complex architecture of the human cerebral cortex [19].
  • DCX immunoreactivity was apparently reduced in ZS, particularly in the cortical plate of fetuses, and in the subependymal foci of heterotopic neurons of the infants [20].
  • Both LIS1 and DCX participate in the development of GABAergic interneurons as well as pyramidal neurons, while ARX participates only in that of interneurons [21].

Associations of DCX with chemical compounds

  • DCX immunocytochemistry showed that immature hippocampal cells of rats exposed to cocaine displayed normal arborization patterns and similar degrees of colocalization with BrdU at two different developmental stages [22].
  • The rate of adult neurogenesis is doubled in the dentate gyrus of Bcl-2-overexpressing mice as demonstrated by quantification of progenitor cells using DCX and new neurons using bromodeoxyuridine (BrdU)/neuronal nuclei antigen (NeuN) double-labelling [23].
  • Following induction of lesions by striatal injection of 6-OHDA or QA, the number of cells positive for doublecortin (DCX) was strongly increased in the striatum; however, there was no change in the number of DCX-positive cells following 6-OHDA injection into the SN [24].
  • The data also suggest that the synapses on DCX-labeled hilar basal dendrites contribute to the persistence of hilar basal dendrites on neurons born after pilocarpine-induced seizures [25].
  • DCX was purified by preparative thin layer chromatography from chloroform: methanol = 4:1 extracts of whole bacteria, and is chromatographically homogeneous [26].

Physical interactions of DCX

  • Furthermore, endogenous DCX could be co-immunoprecipitated with endogenous LIS1 in embryonic brain extracts, demonstrating an in vivo association [27].

Regulatory relationships of DCX

  • Epitope-tagged DCX transiently expressed in COS cells can be co-immunoprecipitated with endogenous LIS1 [27].
  • During brain development DCLK is expressed mainly in postmigratory neurons in a similar pattern to DCX [28].

Other interactions of DCX

  • The distinct LIS patterns suggest that LIS1 and XLIS may be part of overlapping, but distinct, signaling pathways that promote neuronal migration [16].
  • We expressed DCX or DCLK (KIAA0369) repeats in vitro and in vivo [14].
  • A YAC contig in Xq22.3-q23, from DXS287 to DXS8088, spanning the brain-specific genes doublecortin (DCX) and PAK3 [29].
  • Missense DCX mutations may manifest as non-syndromic mental retardation with cryptogenic epilepsy in female subjects and SBH in boys [5].
  • The extensive co-localization of DCX and Calretinin in non-radially oriented neurons suggested a non-pyramidal phenotype [30].

Analytical, diagnostic and therapeutic context of DCX


  1. The doublecortin gene, a new molecular marker to detect minimal residual disease in neuroblastoma. Oltra, S., Martinez, F., Orellana, C., Grau, E., Fernandez, J.M., Cañete, A., Castel, V. Diagn. Mol. Pathol. (2005) [Pubmed]
  2. Ectopic doublecortin gene expression suppresses the malignant phenotype in glioblastoma cells. Santra, M., Zhang, X., Santra, S., Jiang, F., Chopp, M. Cancer Res. (2006) [Pubmed]
  3. Relationship between cytochrome 3A activity, inflammatory status and the risk of docetaxel-induced febrile neutropenia: a prospective study. Alexandre, J., Rey, E., Girre, V., Grabar, S., Tran, A., Montheil, V., Rabillon, F., Dieras, V., Jullien, V., Hérait, P., Pons, G., Treluyer, J.M., Goldwasser, F. Ann. Oncol. (2007) [Pubmed]
  4. Doublecortin expression levels in adult brain reflect neurogenesis. Couillard-Despres, S., Winner, B., Schaubeck, S., Aigner, R., Vroemen, M., Weidner, N., Bogdahn, U., Winkler, J., Kuhn, H.G., Aigner, L. Eur. J. Neurosci. (2005) [Pubmed]
  5. Nonsyndromic mental retardation and cryptogenic epilepsy in women with doublecortin gene mutations. Guerrini, R., Moro, F., Andermann, E., Hughes, E., D'Agostino, D., Carrozzo, R., Bernasconi, A., Flinter, F., Parmeggiani, L., Volzone, A., Parrini, E., Mei, D., Jarosz, J.M., Morris, R.G., Pratt, P., Tortorella, G., Dubeau, F., Andermann, F., Dobyns, W.B., Das, S. Ann. Neurol. (2003) [Pubmed]
  6. Transcranial direct current stimulation during sleep improves declarative memory. Marshall, L., Mölle, M., Hallschmid, M., Born, J. J. Neurosci. (2004) [Pubmed]
  7. Mutations in a novel retina-specific gene cause autosomal dominant retinitis pigmentosa. Sullivan, L.S., Heckenlively, J.R., Bowne, S.J., Zuo, J., Hide, W.A., Gal, A., Denton, M., Inglehearn, C.F., Blanton, S.H., Daiger, S.P. Nat. Genet. (1999) [Pubmed]
  8. A novel lysosome-associated membrane glycoprotein, DC-LAMP, induced upon DC maturation, is transiently expressed in MHC class II compartment. de Saint-Vis, B., Vincent, J., Vandenabeele, S., Vanbervliet, B., Pin, J.J., Aït-Yahia, S., Patel, S., Mattei, M.G., Banchereau, J., Zurawski, S., Davoust, J., Caux, C., Lebecque, S. Immunity (1998) [Pubmed]
  9. A neuronal receptor, neuropilin-1, is essential for the initiation of the primary immune response. Tordjman, R., Lepelletier, Y., Lemarchandel, V., Cambot, M., Gaulard, P., Hermine, O., Roméo, P.H. Nat. Immunol. (2002) [Pubmed]
  10. The multipolar stage and disruptions in neuronal migration. LoTurco, J.J., Bai, J. Trends Neurosci. (2006) [Pubmed]
  11. Hyperventilation-induced EEG changes in humans and their modulation by an anticonvulsant drug. Rockstroh, B. Epilepsy Res. (1990) [Pubmed]
  12. Bone marrow failure syndromes in children. Alter, B.P. Pediatr. Clin. North Am. (2002) [Pubmed]
  13. Occurrence and antigenic heterogeneity of L-2,4-diaminobutyrate decarboxylase in Acinetobacter species. Yamamoto, S., Ikai, H., Uesugi, T., Horie, A., Hirai, Y. Biol. Pharm. Bull. (1995) [Pubmed]
  14. Doublecortin mutations cluster in evolutionarily conserved functional domains. Sapir, T., Horesh, D., Caspi, M., Atlas, R., Burgess, H.A., Wolf, S.G., Francis, F., Chelly, J., Elbaum, M., Pietrokovski, S., Reiner, O. Hum. Mol. Genet. (2000) [Pubmed]
  15. Mutation analysis of the DCX gene and genotype/phenotype correlation in subcortical band heterotopia. Matsumoto, N., Leventer, R.J., Kuc, J.A., Mewborn, S.K., Dudlicek, L.L., Ramocki, M.B., Pilz, D.T., Mills, P.L., Das, S., Ross, M.E., Ledbetter, D.H., Dobyns, W.B. Eur. J. Hum. Genet. (2001) [Pubmed]
  16. LIS1 and XLIS (DCX) mutations cause most classical lissencephaly, but different patterns of malformation. Pilz, D.T., Matsumoto, N., Minnerath, S., Mills, P., Gleeson, J.G., Allen, K.M., Walsh, C.A., Barkovich, A.J., Dobyns, W.B., Ledbetter, D.H., Ross, M.E. Hum. Mol. Genet. (1998) [Pubmed]
  17. High expression of doublecortin and KIAA0369 protein in fetal brain suggests their specific role in neuronal migration. Mizuguchi, M., Qin, J., Yamada, M., Ikeda, K., Takashima, S. Am. J. Pathol. (1999) [Pubmed]
  18. The DCX-domain tandems of doublecortin and doublecortin-like kinase. Kim, M.H., Cierpicki, T., Derewenda, U., Krowarsch, D., Feng, Y., Devedjiev, Y., Dauter, Z., Walsh, C.A., Otlewski, J., Bushweller, J.H., Derewenda, Z.S. Nat. Struct. Biol. (2003) [Pubmed]
  19. Identification of neurabin II as a novel doublecortin interacting protein. Tsukada, M., Prokscha, A., Oldekamp, J., Eichele, G. Mech. Dev. (2003) [Pubmed]
  20. A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. Qin, J., Mizuguchi, M., Itoh, M., Takashima, S. Acta Neuropathol. (2000) [Pubmed]
  21. A new paradigm for West syndrome based on molecular and cell biology. Kato, M. Epilepsy Res. (2006) [Pubmed]
  22. Chronic cocaine exposure impairs progenitor proliferation but spares survival and maturation of neural precursors in adult rat dentate gyrus. Domínguez-Escribà, L., Hernández-Rabaza, V., Soriano-Navarro, M., Barcia, J.A., Romero, F.J., García-Verdugo, J.M., Canales, J.J. Eur. J. Neurosci. (2006) [Pubmed]
  23. Increased generation of granule cells in adult Bcl-2-overexpressing mice: a role for cell death during continued hippocampal neurogenesis. Kuhn, H.G., Biebl, M., Wilhelm, D., Li, M., Friedlander, R.M., Winkler, J. Eur. J. Neurosci. (2005) [Pubmed]
  24. Environmental enrichment brings a beneficial effect on beam walking and enhances the migration of doublecortin-positive cells following striatal lesions in rats. Urakawa, S., Hida, H., Masuda, T., Misumi, S., Kim, T.S., Nishino, H. Neuroscience (2007) [Pubmed]
  25. Newly born dentate granule neurons after pilocarpine-induced epilepsy have hilar basal dendrites with immature synapses. Shapiro, L.A., Ribak, C.E. Epilepsy Res. (2006) [Pubmed]
  26. Cytotoxicity of a novel lipid-like bacterial product. Keler, T., Kovats, E., Nguyen, V., Samu, J., Sanavi, F., Somlyo, B., Nowotny, A. Biochem. Biophys. Res. Commun. (1987) [Pubmed]
  27. Interaction between LIS1 and doublecortin, two lissencephaly gene products. Caspi, M., Atlas, R., Kantor, A., Sapir, T., Reiner, O. Hum. Mol. Genet. (2000) [Pubmed]
  28. Doublecortin-like kinase is associated with microtubules in neuronal growth cones. Burgess, H.A., Reiner, O. Mol. Cell. Neurosci. (2000) [Pubmed]
  29. A YAC contig in Xq22.3-q23, from DXS287 to DXS8088, spanning the brain-specific genes doublecortin (DCX) and PAK3. Allen, K.M., Gleeson, J.G., Shoup, S.M., Walsh, C.A. Genomics (1998) [Pubmed]
  30. Selective expression of doublecortin and LIS1 in developing human cortex suggests unique modes of neuronal movement. Meyer, G., Perez-Garcia, C.G., Gleeson, J.G. Cereb. Cortex (2002) [Pubmed]
  31. Neocortical neuronal arrangement in LIS1 and DCX lissencephaly may be different. Viot, G., Sonigo, P., Simon, I., Simon-Bouy, B., Chadeyron, F., Beldjord, C., Tantau, J., Martinovic, J., Esculpavit, C., Brunelle, F., Munnich, A., Vekemans, M., Encha-Razavi, F. Am. J. Med. Genet. A (2004) [Pubmed]
  32. Genetic alteration of the DCX gene in Japanese patients with subcortical laminar heterotopia or isolated lissencephaly sequence. Sakamoto, M., Ono, J., Okada, S., Nakamura, Y., Kurahashi, H. J. Hum. Genet. (2000) [Pubmed]
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