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Gene Review

PLAGL1  -  pleiomorphic adenoma gene-like 1

Homo sapiens

Synonyms: LOT-1, LOT1, Lost on transformation 1, Pleiomorphic adenoma-like protein 1, Tumor suppressor ZAC, ...
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Disease relevance of PLAGL1


High impact information on PLAGL1


Biological context of PLAGL1

  • In contrast, PLAGL1 has been shown to prevent the proliferation of tumor cells by inducing cell cycle arrest and apoptosis [8].
  • Together with PLAGL1 and PLAGL2, PLAG1 belongs to a subfamily of C(2)H(2) zinc finger transcription factors that activate transcription through binding to the bipartite consensus sequence GRGGC(N)(6-8)GGG [9].
  • Genome mapping of LOT1 in comparison with the other splice variants, namely ZAC1 and PLAGL1, revealed that its mRNA ( approximately 4.7 kb; GenBank accession number U76261) is potentially spliced using six exons spanning at least 70 kb of the human genome [10].
  • We now report that a transgene carrying the human HYMAI/PLAGL1 DMR was methylated in the correct parent-origin-specific manner in mice and this was sufficient to confer imprinted expression from the transgene [11].
  • We conclude that down-regulation of PLAGL1 may be a significant contributing factor in the development of EMC tumors [1].

Anatomical context of PLAGL1

  • A differential display analysis has identified the PLAGL1 gene as being down-regulated in the CFK2(EWS/NOR1) cell line compared to native CFK2 cells [1].
  • RT-PCR analyses show that whereas the PLAGL1 mRNAs encoding the two isoforms of the protein are highly expressed in four human chondrocyte immortalized cell lines and two human chondrocyte primary cultures, they are strongly down-regulated in six EMC tumors [1].
  • Collectively, these results indicate that PLAGL1 is not likely to be the major target gene of the 6q rearrangements in salivary gland tumors [3].
  • We previously reported cloning the rLot1 gene, and its human homolog (hLOT1), through analysis of differential gene expression in normal and malignant rat ovarian surface epithelial cells [12].
  • The enhancement of p53 activity by Zac1 was much more dramatic in HeLa cells than in other cell lines tested [13].

Associations of PLAGL1 with chemical compounds


Physical interactions of PLAGL1


Regulatory relationships of PLAGL1

  • ZAC induces LIT1 transcription in a methylation-dependent manner [2].

Other interactions of PLAGL1


Analytical, diagnostic and therapeutic context of PLAGL1


  1. The PLAGL1 gene is down-regulated in human extraskeletal myxoid chondrosarcoma tumors. Poulin, H., Labelle, Y. Cancer Lett. (2005) [Pubmed]
  2. ZAC, LIT1 (KCNQ1OT1) and p57KIP2 (CDKN1C) are in an imprinted gene network that may play a role in Beckwith-Wiedemann syndrome. Arima, T., Kamikihara, T., Hayashida, T., Kato, K., Inoue, T., Shirayoshi, Y., Oshimura, M., Soejima, H., Mukai, T., Wake, N. Nucleic Acids Res. (2005) [Pubmed]
  3. Molecular analyses of the candidate tumor suppressor gene, PLAGL1, in benign and malignant salivary gland tumors. Enlund, F., Persson, F., Stenman, G. Eur. J. Oral Sci. (2004) [Pubmed]
  4. Establishment of the primary imprint of the HYMAI/PLAGL1 imprint control region during oogenesis. Arima, T., Wake, N. Cytogenet. Genome Res. (2006) [Pubmed]
  5. Regulation of apoptosis and cell cycle arrest by Zac1, a novel zinc finger protein expressed in the pituitary gland and the brain. Spengler, D., Villalba, M., Hoffmann, A., Pantaloni, C., Houssami, S., Bockaert, J., Journot, L. EMBO J. (1997) [Pubmed]
  6. hZAC encodes a zinc finger protein with antiproliferative properties and maps to a chromosomal region frequently lost in cancer. Varrault, A., Ciani, E., Apiou, F., Bilanges, B., Hoffmann, A., Pantaloni, C., Bockaert, J., Spengler, D., Journot, L. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  7. Genome-wide allelotyping of a new in vitro model system reveals early events in breast cancer progression. Li, Z., Meng, Z.H., Sayeed, A., Shalaby, R., Ljung, B.M., Dairkee, S.H. Cancer Res. (2002) [Pubmed]
  8. The tumorigenic diversity of the three PLAG family members is associated with different DNA binding capacities. Hensen, K., Van Valckenborgh, I.C., Kas, K., Van de Ven, W.J., Voz, M.L. Cancer Res. (2002) [Pubmed]
  9. Repression of the Transactivating Capacity of the Oncoprotein PLAG1 by SUMOylation. Van Dyck, F., Delvaux, E.L., Van de Ven, W.J., Chavez, M.V. J. Biol. Chem. (2004) [Pubmed]
  10. LOT1 (PLAGL1/ZAC1), the candidate tumor suppressor gene at chromosome 6q24-25, is epigenetically regulated in cancer. Abdollahi, A., Pisarcik, D., Roberts, D., Weinstein, J., Cairns, P., Hamilton, T.C. J. Biol. Chem. (2003) [Pubmed]
  11. The human HYMAI/PLAGL1 differentially methylated region acts as an imprint control region in mice. Arima, T., Yamasaki, K., John, R.M., Kato, K., Sakumi, K., Nakabeppu, Y., Wake, N., Kono, T. Genomics (2006) [Pubmed]
  12. LOT1 is a growth suppressor gene down-regulated by the epidermal growth factor receptor ligands and encodes a nuclear zinc-finger protein. Abdollahi, A., Bao, R., Hamilton, T.C. Oncogene (1999) [Pubmed]
  13. Enhancement of p53-dependent gene activation by the transcriptional coactivator Zac1. Huang, S.M., Schönthal, A.H., Stallcup, M.R. Oncogene (2001) [Pubmed]
  14. Identification of a zinc-finger gene at 6q25: a chromosomal region implicated in development of many solid tumors. Abdollahi, A., Roberts, D., Godwin, A.K., Schultz, D.C., Sonoda, G., Testa, J.R., Hamilton, T.C. Oncogene (1997) [Pubmed]
  15. The candidate tumor suppressor gene ZAC is involved in keratinocyte differentiation and its expression is lost in basal cell carcinomas. Basyuk, E., Coulon, V., Le Digarcher, A., Coisy-Quivy, M., Moles, J.P., Gandarillas, A., Journot, L. Mol. Cancer Res. (2005) [Pubmed]
  16. Multicenter clinical trial of zinc acexamate in the prevention of nonsteroidal antiinflammatory drug induced gastroenteropathy. Spanish Study Group on NSAID Induced Gastroenteropathy Prevention. Rodríguez de la Serna, A., Díaz-Rubio, M. J. Rheumatol. (1994) [Pubmed]
  17. ZAC1 is up-regulated by hypertonicity and decreases sorbitol dehydrogenase expression, allowing accumulation of sorbitol in kidney cells. Lanaspa, M.A., Andres-Hernando, A., Rivard, C.J., Dai, Y., Li, N., Berl, T. J. Biol. Chem. (2009) [Pubmed]
  18. Involvement of PLAGL2 in activation of iron deficient- and hypoxia-induced gene expression in mouse cell lines. Furukawa, T., Adachi, Y., Fujisawa , J., Kambe, T., Yamaguchi-Iwai, Y., Sasaki, R., Kuwahara, J., Ikehara, S., Tokunaga, R., Taketani, S. Oncogene (2001) [Pubmed]
  19. Chromosome 6 deletion and candidate tumor suppressor genes in adenoid cystic carcinoma. Rutherford, S., Yu, Y., Rumpel, C.A., Frierson, H.F., Moskaluk, C.A. Cancer Lett. (2006) [Pubmed]
  20. Induction of type I PACAP receptor expression by the new zinc finger protein Zac1 and p53. Hoffmann, A., Ciani, E., Houssami, S., Brabet, P., Journot, L., Spengler, D. Ann. N. Y. Acad. Sci. (1998) [Pubmed]
  21. Neonatal diabetes, with hypoplastic pancreas, intestinal atresia and gall bladder hypoplasia: search for the aetiology of a new autosomal recessive syndrome. Mitchell, J., Punthakee, Z., Lo, B., Bernard, C., Chong, K., Newman, C., Cartier, L., Desilets, V., Cutz, E., Hansen, I.L., Riley, P., Polychronakos, C. Diabetologia (2004) [Pubmed]
  22. Epigenetic silencing of the imprinted gene ZAC by DNA methylation is an early event in the progression of human ovarian cancer. Kamikihara, T., Arima, T., Kato, K., Matsuda, T., Kato, H., Douchi, T., Nagata, Y., Nakao, M., Wake, N. Int. J. Cancer (2005) [Pubmed]
  23. Altered expression and loss of heterozygosity of the LOT1 gene in ovarian cancer. Cvetkovic, D., Pisarcik, D., Lee, C., Hamilton, T.C., Abdollahi, A. Gynecol. Oncol. (2004) [Pubmed]
  24. Loss of expression of the candidate tumor suppressor gene ZAC in breast cancer cell lines and primary tumors. Bilanges, B., Varrault, A., Basyuk, E., Rodriguez, C., Mazumdar, A., Pantaloni, C., Bockaert, J., Theillet, C., Spengler, D., Journot, L. Oncogene (1999) [Pubmed]
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