Recombinant Human Zinc finger protein PLAG1 (PLAG1)

1. Rearrangement of PLAG1 and HMGA2 and expression of the corresponding proteins are frequent and specific findings in lacrimal gland pleomorphic adenoma (PA) and ca-ex-PA. PMID: 29437290
2. PLAG1 is a microRNA target gene that is overexpressed in Wilms tumors with mutations in microRNA processing genes. PMID: 30026293
3. HRAS mutations were more common in epithelial-myoepithelial carcinomas (EMCAs) with intact PLAG1 and HMGA2. Most EMCAs arose ex pleomorphic adenoma (PA)and the genetic profile of EMCA varies with the absence or presence of preexisting PA and its cytogenetic signature. Progression to higher grade EMCA with intact PLAG1 and HMGA2 correlates with the presence of TP53, FBXW7 mutations, or SMARCB1 deletion. PMID: 29135520
4. Plag1 expression is lost in malignant transformation from pleomorphic adenoma to carcinoma ex pleomorphic adenoma. PMID: 27473265
5. MiR-26a can facilitate occurrence of pituitary tumor and invasiveness, probably via inhibiting PLAG1 expression. PMID: 28012286
6. expression of PLAG1 can be considered a valuable diagnostic marker for recurrent pleomorphic adenoma PMID: 27381214
7. High PLAG1 expression is associated with pleomorphic adenoma. PMID: 26882287
8. The sensitivity (55%) and specificity (75%) of PLAG1 in diagnosing PA in FNAs is relatively modest thus limiting its diagnostic utility. PMID: 27463119
9. The Thr34 of PLAG1 is critical for LpMab-10 recognition. PMID: 26492619
10. Data indicate that SLIT2, miR-218-1, RET/PLAG1 and SLIT2/ROBO1 pathway involved in Hirschsprung's disease. PMID: 25786906
11. Altogether, these data suggest that HMGA2 is an upstream activator of PLAG1. PMID: 24516594
12. Report illustrates two different tumorigenic pathways implicating PLAG1 in lipoblastoma: amplification through multiple copies of a small marker chromosome derived from chromosome 8, and a paracentric inversion of the long arm of chromosome 8. Review. PMID: 24433523
13. The nuclear import of PLAG1 by KPNA2 is essential for the role of KPNA2 in HCC cells. PMID: 25060425
14. a marker with good specificity for salivary gland pleomorphic adenomas PMID: 23958548
15. Extraskeletal myxoid chondrosarcoma of the vulva with PLAG1 gene activation: molecular genetic characterization of 2 cases PMID: 24185117
16. Fluorescence in situ hybridization for PLAG1 or HMGA2 can be used to distinguish between pleomorphic adenoma and carcinoma ex-pleomorphic adenoma and their morphologic mimics. PMID: 25439740
17. lipoblastoma is a group of lipomatous tumors with PLAG1 rearrangement and overexpression. PMID: 24700772
18. These results suggest that PLAG1 and CYLD do not play a role in ACC tumorigenesis. PMID: 23404581
19. Lacrimal and salivary gland PAs and Ca-ex-PAs have similar genomic profiles and frequently overexpress the PLAG1 oncoprotein. Copy number gains involving 9p23-p22.3 (NFIB) and 22q12-qter (PDGFB) may be of importance for disease progression. PMID: 24468654
20. Ten cases of salivary duct carcinoma had PLAG1 rearrangement/amplification (22.7%) and eight had HMGA2 (18.2%) rearrangement/amplification. PMID: 23738717
21. PLAG1 not only activates genes that promote cell proliferation and tumor formation but also genes that inhibit these cellular processes. PMID: 23690029
22. A subset of cutaneous and soft tissue ME tumors appears genetically linked to their salivary gland counterparts, displaying frequent PLAG1 gene rearrangements and occasionally LIFR-PLAG1 fusion. PMID: 23630011
23. miR-141 contributes to fetal growth restriction by regulating PLAG1 expression. PMID: 23554918
24. PLAG1 binding to the IGF2 P3 promoter and IGF2 expression is cell type-specific, and that the PLAG1 transcription factor acts as a transcriptional facilitator that partially overrides the insulation by the H19 ICR. PMID: 23023303
25. We conclude that most Carcinoma ex pleomorphic adenoma, regardless of morphologic subtype,carry altered PLAG1 genes PMID: 22485045
26. PLAG1 is evidently not involved in the development of myoepithelial tumours. The proportion of 8q12-alterations in myoepithelial tumours was very low. PMID: 22593475
27. PLAG1 immunohistochemistry is useful for distinguishing lipoblastoma from other lipomatous tumors including liposarcoma. PMID: 22192798
28. myoepithelial tumors with PLAG1 alteration share a common morphologic phenotype with salivary gland-like morphology and are genetically related to their salivary gland counterpart PMID: 22038920
29. close relationship between cutaneous mixed tumors and pleomorphic adenomas of the salivary gland. However, the mechanism of PLAG1 expression in cutaneous mixed tumors appears to be possibly different from that of pleomorphic adenomas. PMID: 21927843
30. present results also suggest that overexpression of PLAG1 is essential for the tumorigenesis of pleomorphic adenomas, although the mechanisms mediating PLAG1 overexpression seem to be variable. PMID: 21394649
31. Overexpression of the oncogene PLAG1 is associated with the pathogenesis of chronic lymphocytic leukemia. PMID: 20687796
32. Overexpression in transgenic mice causes hypereinsulinemic normoglycemia and insulin resistance. PMID: 20522588
33. Identification of a karyopherin alpha 2 recognition site in PLAG1, which functions as a nuclear localization signal PMID: 11882654
34. overexpression of PLAG1 does not depend on chromosomal rearrangements in radiation associated pleomorphic adenomas PMID: 11894114
35. PLAG1 regulates promoter P3-dependent transcription of IGF2 in hepatoblastomas. PMID: 14695992
36. a direct repression of the transactivating capacity of the oncoprotein PLAG1 by SUMOylation PMID: 15208321
37. PLAG1 amplification is associated with malignant mixed tumor of salivary gland PMID: 15262430
38. fusion with HAS2 in lipoblastoma PMID: 15642402
39. Finding reinforces the role of PLAG1 on the tumorigenesis of benign and malignant pleomorphic adenoma. PMID: 15920557
40. Results establish the in vivo tumorigenic capacity of PLAG1 and indicate that the transgenic mice constitute a valuable model for pleomorphic salivary gland tumorigenesis and potentially for other glands as well. PMID: 15930271
41. Overexpression of PLAG1 is associated with pleomorphic adenomas in transgenic mice PMID: 16108035
42. PLAG1 protein is overexpressed in epithelial, myoepithelial, and mesenchymal-like tumor cells in tumors with fusions to CHCHD7 and TCEA1. PMID: 16736500
43. The importance and versatility of the PLAG1 oncogene in tumourigenesis is discussed. PMID: 17332914
44. PLAG1 gene rearrangements is associated with pleomorphic adenoma PMID: 17693184
45. Molecular analyses in salivary gland tumors revealed that ring formation consistently generated novel FGFR1-PLAG1 gene fusions in which the 5'-part of FGFR1 is linked to the coding sequence of PLAG1 PMID: 18059337
46. Fluorescence in situ hybridization (FISH) demonstrated rearrangements of the PLAG1 region in lipoblastoma in adolescencts and young adults. PMID: 18269579
47. overexpression of PLAG1 is associated with pleomorphic adenomas of the salivary gland. PMID: 19347935
48. a significant regulation of PLAG1 by miR-181a, miR-181b, miR-107, and miR-424. PMID: 19692702

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A chromosomal aberration involving PLAG1 is found in salivary gland pleiomorphic adenomas, the most common benign epithelial tumors of the salivary gland. Translocation t(3;8)(p21;q12) with constitutively expressed beta-catenin/CTNNB1. Fusion occurs in the 5'-regulatory regions, leading to promoter swapping between the 2 genes and activation of PLAG1 expression in adenomas. The chimeric transcript is formed by fusion of CTNNB1 exon 1 to PLAG1 exon 3. Reciprocal fusion transcript consisting of PLAG1 exon 1 and CTNNB1 exon 2-16 is also revealed in some adenomas (PubMed:9020842, PubMed:10029085). Translocation t(3;8)(p21;q12) with transcription elongation factor SII/TCEA1. The fusion transcript is composed of 5'-non-coding sequences as well as 63 nucleotides of the coding region of TCEA1 fused to the acceptor splice site of PLAG1 exon 3. The fusion transcript encodes a truncated TCEA1-PLAG1 protein of 90 AA as well as an apparently normal PLAG1 protein. Reciprocal fusion transcript PLAG1-TCEA1 is also present in one adenoma (PubMed:10029085, PubMed:16736500). Translocation t(5;8)(p13;q12) with leukemia inhibitory factor receptor LIFR. This fusion occured in the 5'-non-coding sequences of both genes, exchanging regulatory control element while preserving the coding sequences (PubMed:9525740). Translocation t(6;8)(p21.3-22;q13) with Coiled-coil-helix-coiled-coil-helix domain-containing protein 7/CHCHD7. Fusion occurs in the 5' regulatory regions, leading to promoter swapping and up-regulation of PLAG1 expression (PubMed:16736500). Ectopic expression of PLAG1 under the control of promoters of distinct translocation partner genes is a general pathogenetic mechanism for pleiomorphic adenomas with 8q aberrations. These fusion genes are likely to be found in adenomas with normal karyotype as this subgroup of tumors also exhibit PLAG1 activation (PubMed:9020842, PubMed:10029085, PubMed:9525740, PubMed:16736500).

Nucleus. Note=Strong nucleolar localization when sumoylation is inhibited.

Krueppel C2H2-type zinc-finger protein family

Expressed in fetal tissues such as lung, liver and kidney. Not detected or weak detection in normal adult tissues, but highly expressed in salivary gland with benign or malignant pleiomorphic adenomas with or without 8q12 aberrations, with preferential oc

HGNC: 9045

OMIM: 181030

KEGG: hsa:5324

STRING: 9606.ENSP00000325546

UniGene: Hs.14968