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Gene Review

DDX1  -  DEAD (Asp-Glu-Ala-Asp) box helicase 1

Homo sapiens

Synonyms: ATP-dependent RNA helicase DDX1, DBP-RB, DEAD box protein 1, DEAD box protein retinoblastoma, UKVH5d
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Disease relevance of DDX1


High impact information on DDX1

  • DEAD box proteins, characterized by the conserved motif Asp-Glu-Ala-Asp, are putative RNA helicases implicated in a number of cellular processes involving alteration of RNA secondary structure such as translation initiation, nuclear and mitochondrial splicing, and ribosome and spliceosome assembly [5].
  • No evidence for correlation of DDX1 gene amplification with improved survival probability in patients with MYCN-amplified neuroblastomas [6].
  • Coamplification of DDX1 correlates with an improved survival probability in children with MYCN-amplified human neuroblastoma [7].
  • Coamplification is correlated to an upregulated gene expression for DDX1 and NAG [7].
  • Although inhibitors of RNA transcription had no effect on DDX1 bodies or cleavage bodies, inhibitors of DNA replication resulted in loss of CstF-64-containing cleavage bodies [8].

Biological context of DDX1

  • By microscopic fluorescence resonance energy transfer, we show that labeled DDX1 resides within a Förster distance of 10 nm of labeled CstF-64 protein in both the nucleoplasm and within cleavage bodies [9].
  • We determined the gene copy number of DDX1 in 32 neuroblastoma patient samples (representative of all stages): 13 were MYCN amplified and 19 had normal copy numbers of the MYCN gene [2].
  • All four nuclear bodies associate with each other during S phase, with cleavage bodies colocalizing with DDX1 bodies, and cleavage bodies/DDX1 bodies residing adjacent to gems and CBs [8].
  • In addition to the eight conserved motifs that characterize all DEAD box proteins, the deduced amino acid sequence of DDX1 contains a subregion with considerable homology to heterogeneous nuclear ribonucleoprotein U [10].
  • DEAD box proteins are putative RNA helicases that function in all aspects of RNA metabolism, including translation, ribosome biogenesis, and pre-mRNA splicing [9].

Anatomical context of DDX1

  • DDX1, however was found to be amplified along with N-myc in 4/6 (67%) cell lines and 6/16 (38%) of the N-myc amplified tumours [11].
  • We conclude that altered DDX1 expression in human astrocytes is, at least in part, responsible for the unfavorable cellular microenvironment for Rev function in these CNS-based cells [12].

Physical interactions of DDX1

  • Coimmunoprecipitation analysis indicates that a proportion of CstF-64 protein resides in the same complex as DDX1 [9].

Other interactions of DDX1

  • These observations indicate that there is at least 1 other gene co-amplified with MYCN in a proportion of cases and that those patients with DDX1 co-amplification tend to relapse more quickly [13].
  • An RNA helicase, DDX1, interacting with poly(A) RNA and heterogeneous nuclear ribonucleoprotein K [14].
  • Three identified proteins (PCBP2, G3BP1, and DDX1) were selected for further investigation into their possible roles on the HCV replication [15].

Analytical, diagnostic and therapeutic context of DDX1


  1. Expression of two dead box genes (DDX1 and DDX6) is independent of that of MYCN in human neuroblastoma cell lines. Akiyama, K., Akao, Y., Yokoyama, M., Nakagawa, Y., Noguchi, T., Yagi, K., Nishi, Y. Biochem. Mol. Biol. Int. (1999) [Pubmed]
  2. Co-amplification of MYCN and a DEAD box gene (DDX1) in primary neuroblastoma. Squire, J.A., Thorner, P.S., Weitzman, S., Maggi, J.D., Dirks, P., Doyle, J., Hale, M., Godbout, R. Oncogene (1995) [Pubmed]
  3. A DEAD box protein facilitates HIV-1 replication as a cellular co-factor of Rev. Fang, J., Kubota, S., Yang, B., Zhou, N., Zhang, H., Godbout, R., Pomerantz, R.J. Virology (2004) [Pubmed]
  4. Reduced levels of DEAD-box proteins DBP-RB and p72 in fetal Down syndrome brains. Kircher, S.G., Kim, S.H., Fountoulakis, M., Lubec, G. Neurochem. Res. (2002) [Pubmed]
  5. Amplification of a DEAD box protein gene in retinoblastoma cell lines. Godbout, R., Squire, J. Proc. Natl. Acad. Sci. U.S.A. (1993) [Pubmed]
  6. No evidence for correlation of DDX1 gene amplification with improved survival probability in patients with MYCN-amplified neuroblastomas. De Preter, K., Speleman, F., Combaret, V., Lunec, J., Board, J., Pearson, A., De Paepe, A., Van Roy, N., Laureys, G., Vandesompele, J. J. Clin. Oncol. (2005) [Pubmed]
  7. Coamplification of DDX1 correlates with an improved survival probability in children with MYCN-amplified human neuroblastoma. Weber, A., Imisch, P., Bergmann, E., Christiansen, H. J. Clin. Oncol. (2004) [Pubmed]
  8. Dynamic nature of cleavage bodies and their spatial relationship to DDX1 bodies, Cajal bodies, and gems. Li, L., Roy, K., Katyal, S., Sun, X., Bléoo, S., Godbout, R. Mol. Biol. Cell (2006) [Pubmed]
  9. Association of human DEAD box protein DDX1 with a cleavage stimulation factor involved in 3'-end processing of pre-MRNA. Bléoo, S., Sun, X., Hendzel, M.J., Rowe, J.M., Packer, M., Godbout, R. Mol. Biol. Cell (2001) [Pubmed]
  10. A human DEAD box protein with partial homology to heterogeneous nuclear ribonucleoprotein U. Godbout, R., Hale, M., Bisgrove, D. Gene (1994) [Pubmed]
  11. Investigation of co-amplification of the candidate genes ornithine decarboxylase, ribonucleotide reductase, syndecan-1 and a DEAD box gene, DDX1, with N-myc in neuroblastoma. United Kingdom Children's Cancer Study Group. George, R.E., Kenyon, R.M., McGuckin, A.G., Malcolm, A.J., Pearson, A.D., Lunec, J. Oncogene (1996) [Pubmed]
  12. The RNA helicase DDX1 is involved in restricted HIV-1 Rev function in human astrocytes. Fang, J., Acheampong, E., Dave, R., Wang, F., Mukhtar, M., Pomerantz, R.J. Virology (2005) [Pubmed]
  13. Analysis of candidate gene co-amplification with MYCN in neuroblastoma. George, R.E., Kenyon, R., McGuckin, A.G., Kohl, N., Kogner, P., Christiansen, H., Pearson, A.D., Lunec, J. Eur. J. Cancer (1997) [Pubmed]
  14. An RNA helicase, DDX1, interacting with poly(A) RNA and heterogeneous nuclear ribonucleoprotein K. Chen, H.C., Lin, W.C., Tsay, Y.G., Lee, S.C., Chang, C.J. J. Biol. Chem. (2002) [Pubmed]
  15. Subproteomic analysis of the cellular proteins associated with the 3' untranslated region of the hepatitis C virus genome in human liver cells. Tingting, P., Caiyun, F., Zhigang, Y., Pengyuan, Y., Zhenghong, Y. Biochem. Biophys. Res. Commun. (2006) [Pubmed]
  16. Physical mapping of the DDX1 gene to 340 kb 5' of MYCN. Kuroda, H., White, P.S., Sulman, E.P., Manohar, C.F., Reiter, J.L., Cohn, S.L., Brodeur, G.M. Oncogene (1996) [Pubmed]
  17. The DDX1 gene maps within 400 kbp 5' to MYCN and is frequently coamplified in human neuroblastoma. Amler, L.C., Schürmann, J., Schwab, M. Genes Chromosomes Cancer (1996) [Pubmed]
  18. Quantification of MYCN, DDX1, and NAG gene copy number in neuroblastoma using a real-time quantitative PCR assay. De Preter, K., Speleman, F., Combaret, V., Lunec, J., Laureys, G., Eussen, B.H., Francotte, N., Board, J., Pearson, A.D., De Paepe, A., Van Roy, N., Vandesompele, J. Mod. Pathol. (2002) [Pubmed]
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