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Gene Review

Zic1  -  zinc finger protein of the cerebellum 1

Mus musculus

Synonyms: ZIC, ZNF201, Zic, Zinc finger protein ZIC 1, Zinc finger protein of the cerebellum 1, ...
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Disease relevance of Zic1


High impact information on Zic1

  • Although the Bent tail phenotype probably is the result of the deletion of several genes, combining data on ZIC:3 expression and function of ZIC: genes in the mouse shows that deletion of Zic3 alone is compatible with a major role of this gene in the congenital malformations of the Bent tail mouse [4].
  • Multiple functions of a Zic-like gene in the differentiation of notochord, central nervous system and muscle in Ciona savignyi embryos [5].
  • These findings suggest that Zic1 may determine the cerebellar folial pattern principally via regulation of cell proliferation in the EGL [6].
  • Zic genes encode zinc finger proteins, the expression of which is highly restricted to cerebellar granule cells and their precursors [6].
  • The nucleotide sequence of human Zic cDNA is 85% homologous to that of mouse Zic cDNA [2].

Biological context of Zic1


Anatomical context of Zic1

  • However, the disruption of Zic1, a strong homolog of Zic2 that has an overlapping expression pattern, results in cerebellar malformation with no apparent abnormalities in the forebrain or in posterior neuropore closure [11].
  • Both Zic1 and Zic2 are expressed in the precursor cells of the granule neuron and the neurons in cerebellar nuclei [11].
  • Our findings suggest that Zic1 and Zic2 have essentially the same properties as Zic3 and that the Xenopus Zic family may act cooperatively in the initial phase of neural and neural crest development [8].
  • In early gastrula, Zic1 expression was restricted to the prospective neural plate region whereas Zic2 was expressed widely in the ectoderm [8].
  • Zic1 promotes the expansion of dorsal neural progenitors in spinal cord by inhibiting neuronal differentiation [9].

Regulatory relationships of Zic1


Other interactions of Zic1

  • We examined the expression of Zic1, Zic2, and Zic3 genes in the mouse embryo by means of in situ hybridization [1].
  • Whereas the embryonic expression patterns of Zic1, 2, 3, and 5 have been described in detail, Zic4 has not yet received close attention [14].
  • The Zic1/Gli3 double mutants showed severe abnormalities of vertebral arches not found in single mutants [12].
  • In the present study, we characterized the fifth member of the mouse Zic family gene, mouse Zic5 [15].
  • The changed expression pattern in Wnt-3a mutant mice suggests that Zic genes in the neural tube are regulated by the factors from notochord [1].

Analytical, diagnostic and therapeutic context of Zic1

  • A novel gene, Dorz1, was identified and characterized as one of the most significantly down-regulated genes in Zic1-deficient cerebellum by the DNA microarray analysis [16].


  1. The expression of the mouse Zic1, Zic2, and Zic3 gene suggests an essential role for Zic genes in body pattern formation. Nagai, T., Aruga, J., Takada, S., Günther, T., Spörle, R., Schughart, K., Mikoshiba, K. Dev. Biol. (1997) [Pubmed]
  2. Predominant expression of human zic in cerebellar granule cell lineage and medulloblastoma. Yokota, N., Aruga, J., Takai, S., Yamada, K., Hamazaki, M., Iwase, T., Sugimura, H., Mikoshiba, K. Cancer Res. (1996) [Pubmed]
  3. The role of Zic genes in neural development. Aruga, J. Mol. Cell. Neurosci. (2004) [Pubmed]
  4. A deletion encompassing Zic3 in bent tail, a mouse model for X-linked neural tube defects. Klootwijk, R., Franke, B., van der Zee, C.E., de Boer, R.T., Wilms, W., Hol, F.A., Mariman, E.C. Hum. Mol. Genet. (2000) [Pubmed]
  5. Multiple functions of a Zic-like gene in the differentiation of notochord, central nervous system and muscle in Ciona savignyi embryos. Imai, K.S., Satou, Y., Satoh, N. Development (2002) [Pubmed]
  6. Mouse Zic1 is involved in cerebellar development. Aruga, J., Minowa, O., Yaginuma, H., Kuno, J., Nagai, T., Noda, T., Mikoshiba, K. J. Neurosci. (1998) [Pubmed]
  7. Identification and characterization of Zic4, a new member of the mouse Zic gene family. Aruga, J., Yozu, A., Hayashizaki, Y., Okazaki, Y., Chapman, V.M., Mikoshiba, K. Gene (1996) [Pubmed]
  8. Xenopus Zic family and its role in neural and neural crest development. Nakata, K., Nagai, T., Aruga, J., Mikoshiba, K. Mech. Dev. (1998) [Pubmed]
  9. Zic1 promotes the expansion of dorsal neural progenitors in spinal cord by inhibiting neuronal differentiation. Aruga, J., Tohmonda, T., Homma, S., Mikoshiba, K. Dev. Biol. (2002) [Pubmed]
  10. Investigation of downstream target genes of PAX3c, PAX3e and PAX3g isoforms in melanocytes by microarray analysis. Wang, Q., Kumar, S., Mitsios, N., Slevin, M., Kumar, P. Int. J. Cancer (2007) [Pubmed]
  11. Zic2 controls cerebellar development in cooperation with Zic1. Aruga, J., Inoue, T., Hoshino, J., Mikoshiba, K. J. Neurosci. (2002) [Pubmed]
  12. Zic1 regulates the patterning of vertebral arches in cooperation with Gli3. Aruga, J., Mizugishi, K., Koseki, H., Imai, K., Balling, R., Noda, T., Mikoshiba, K. Mech. Dev. (1999) [Pubmed]
  13. Myogenic repressor I-mfa interferes with the function of Zic family proteins. Mizugishi, K., Hatayama, M., Tohmonda, T., Ogawa, M., Inoue, T., Mikoshiba, K., Aruga, J. Biochem. Biophys. Res. Commun. (2004) [Pubmed]
  14. Zic4, a zinc-finger transcription factor, is expressed in the developing mouse nervous system. Gaston-Massuet, C., Henderson, D.J., Greene, N.D., Copp, A.J. Dev. Dyn. (2005) [Pubmed]
  15. Mouse Zic5 deficiency results in neural tube defects and hypoplasia of cephalic neural crest derivatives. Inoue, T., Hatayama, M., Tohmonda, T., Itohara, S., Aruga, J., Mikoshiba, K. Dev. Biol. (2004) [Pubmed]
  16. Dorz1, a novel gene expressed in differentiating cerebellar granule neurons, is down-regulated in Zic1-deficient mouse. Hoshino, J., Aruga, J., Ishiguro, A., Mikoshiba, K. Brain Res. Mol. Brain Res. (2003) [Pubmed]
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