Genes (39)
Species:
human : 37 mouse : 2 | |
| Mouse | SPI1 | 6688 | spleen focus forming virus (SFFV) proviral integration oncogene | We have shown that Spi-1 promotes the use of the proximal 5;-splice site during the E1A pre-mRNA splicing and interferes with the effect of TLS (Translocated in LipoSarcoma) in this splicing assay. | | Mouse | FUS | 2521 | fused in sarcoma | Expression of the FUS domain restores liposarcoma development in CHOP transgenic mice. The consequent overexpression of FUS-CHOP results in most of the symptoms of human liposarcomas, including the presence of lipoblasts with round nuclei, accumulation of intracellular lipid, induction of adipocyte-specific genes and a concordant block in the differentiation program. The characteristic t(12;16)(q13;p11) chromosomal translocation, which leads to gene fusion that encodes the FUS-CHOP chimeric protein, is associated with human liposarcomas. We have demonstrated that liposarcomas in the FUS-CHOP transgenic mice express high levels of the adipocyte regulatory protein PPARgamma, whereas it is not expressed in embryonic fibroblasts from these animals following induction to differentiation toward the adipocyte lineage, indicating that the in vitro system does not really reflect the in vivo situation and the developmental defect is downstream of PPARgamma expression. These animals consistently show the accumulation of a glycoprotein material within the terminally differentiated adipocytes, a characteristic figure of liposarcomas associated with FUS-CHOP. On the contrary, transgenic mice expressing the altered form of FUS-CHOP created by the in frame fusion of the FUS domain to the carboxy end of CHOP (CHOP-FUS) developed liposarcomas. In the past, we generated FUS-CHOP and CHOP transgenic mice and have shown that while FUS-CHOP transgenic develop liposarcomas, mice expressing CHOP, which lacks the FUS domain, display essentially normal white adipose tissue (WAT) development, suggesting that the FUS domain of FUS-CHOP plays a specific and critical role in the pathogenesis of liposarcoma. These results provide evidence that the FUS domain of FUS-CHOP plays a specific and critical role in the pathogenesis of liposarcoma. The altered expression of FUS-CHOP has been implicated in a characteristic subgroup of human liposarcomas. The characteristics of the liposarcomas arising in the CHOP-FUS mice were very similar to those previously observed in our FUS-CHOP transgenic mice indicating that the FUS domain is required not only for transformation but also influences the phenotype of the tumor cells. Liposarcoma initiated by FUS/TLS-CHOP: the FUS/TLS domain plays a critical role in the pathogenesis of liposarcoma. Our results provide genetic evidence that FUS and CHOP domains function in trans for the mutual restoration of liposarcoma. Here we report that expression of the FUS domain restores liposarcoma development in CHOP-transgenic mice. | | Human | CREB3L2 | 64764 | cAMP responsive element binding protein 3-like 2 | INFERRED, Score=800, UMLKSK CUI: C0023827 | | Human | DUSP12 | 11266 | dual specificity phosphatase 12 | The human YVH1 gene is located on chromosome 1q21-q22, which falls in a region amplified in human liposarcomas. | | Human | SRSF10 | 10772 | serine/arginine-rich splicing factor 10 | Translocation liposarcoma protein (TLS)-associated serine-arginine (TASR)-1 and -2 are two newly identified serine-arginine splicing factors. Translocation liposarcoma protein (TLS)-associated serine-arginine (TASR)-1 and -2 are two newly identified serine-arginine splicing factors. Characterization and expression of the human gene encoding two translocation liposarcoma protein-associated serine-arginine (TASR) proteins. | | Human | IGF2BP2 | 10644 | insulin-like growth factor 2 mRNA binding protein 2 | we provide evidence that there is a strong and statistically significant correlation between HMGA2 and IMP2 gene expression in human liposarcomas | | Human | MVP | 9961 | major vault protein | The 16p11 breakpoint in myxoid liposarcomas might affect the expression of the LRP gene on 16p11.2 encoding the multidrug resistance associated major vault protein. LRP was strongly expressed in MFH and unspecified sarcomas, but was low in liposarcomas. MATERIALS AND METHODS: Of 16 cases with a diagnosis of a (possible) liposarcoma with an abnormal karyotype, fresh frozen tumour material was available for immunohistological detection of LRP. | | Human | HMGA2 | 8091 | high mobility group AT-hook 2 | In myxoid liposarcomas the translocation t(12;16) creates a fusion gene between the CHOP gene and the FUS gene and in lipomas the HMGI-C gene becomes rearranged by structural aberrations involving chromosomal region 12q14-15. Recent genetic advances, including the ASPL-TFE3 fusion of alveolar soft part sarcoma, the JAZF1-JJAZ1 fusion of endometrial stromal sarcoma, and HMGIC fusions in liposarcoma, are discussed. This truncation of HMGIC resembles that reported for translocations of HMGIC in benign tumors, including lipomas, and it is striking that the gene was frequently amplified or rearranged in well differentiated liposarcomas, the malignant counterpart of lipomas. Ectopic sequences from truncated HMGIC in liposarcomas are derived from various amplified chromosomal regions. We provide evidence that there is a strong and statistically significant correlation between HMGA2 and IMP2 gene expression in human liposarcomas Suppression of HMGA2 protein synthesis could be a tool for the therapy of well differentiated liposarcomas overexpressing HMGA2. In myxoid liposarcomas the translocation t(12;16) creates a fusion gene between the CHOP gene and the FUS gene and in lipomas the HMGI-C gene becomes rearranged by structural aberrations involving chromosomal region 12q14-15. | | Human | WNT1 | 7471 | wingless-type MMTV integration site family, member 1 | INFERRED, Score=800, UMLKSK CUI: C0023827 | | Human | TSN | 7247 | translin | Computer-assisted search found sequences highly homologous (>70%) with Translin binding motifs adjacent to the breakpoints in 10 out of 11 liposarcomas with the TLS-CHOP fusion genes. Translin binds to the sequences adjacent to the breakpoints of the TLS and CHOP genes in liposarcomas with translocation t(12;6). Considering the high incidence and specific binding property, the presence of Translin binding motif may be one of the important determinants for the location of breakpoints in the TLS and CHOP genes in liposarcomas. These data suggested that sequence characteristics may play an important role to recruit several factors such as Translin and topoisomerase II in the process of chromosomal translation in liposarcomas. Characteristics of genomic breakpoints in TLS-CHOP translocations in liposarcomas suggest the involvement of Translin and topoisomerase II in the process of translocation. | | Human | TOP2A | 7153 | topoisomerase (DNA) II alpha 170kDa | Topo-II alpha is a useful marker for diagnosing liposarcoma | | Human | TIMP2 | 7077 | TIMP metallopeptidase inhibitor 2 | Lack of TIMP2 expression was a poor prognostic factor for DFS in synovial sarcoma (P = 0.009), while MMP2 and MMP9 correlated with metastasis (P = 0.008 and P = 0.005, respectively) and grade (P = 0.001 and P = 0.04 respectively) in liposarcoma. | | Human | TERC | 7012 | telomerase RNA component | To examine whether telomerase activity, hTRT and hTR mRNA expression were associated with tumor progression in myxoid liposarcoma, we investigated a total of 28 myxoid liposarcomas (13 pure myxoid tumors, 14 mixed-type tumors, and 1 pure round-cell variant) from 19 patients. HTR mRNA expression was elevated in all 19 liposarcomas. | | Human | TAL1 | 6886 | T-cell acute lymphocytic leukemia 1 | We report here that the N-terminal domains of the proteins TAL1 (ectopically activated in T-cell acute leukemias after chromosomal abnormalities caused by V-D-J recombinase error) (V, variable; D, diversity; J, joining) and FUS-CHOP (a liposarcoma tumor-specific fusion protein that is produced as a result of a chromosomal translocation) can function as transcription activators of specific responsive reporter genes. | | Human | STC1 | 6781 | stanniocalcin 1 | Neoplastic adipocytes in well-differentiated liposarcomas stained for STC1, but the frequency of STC-1-positive cells was lower in high-grade liposarcomas; STC1 may function as a "survival factor", contributing to the maintenance of mature adipose tissue | | Human | SREBF1 | 6720 | sterol regulatory element binding transcription factor 1 | Nelfinavir induces liposarcoma apoptosis and cell cycle arrest by upregulating sterol regulatory element binding protein-1. Our data indicate that nelfinavir represents a novel class of antiliposarcoma agent that acts by selectively upregulating sterol regulatory element binding protein-1 expression in liposarcomas. | | Human | S100A4 | 6275 | S100 calcium binding protein A4 | MATERIALS AND METHODS: In the human liposarcoma cell line, SW872, we analyzed the expression of S100A4 protein by immunohistochemistry and flow cytometry. | | Human | RELA | 5970 | v-rel avian reticuloendotheliosis viral oncogene homolog A | In this study, we have demonstrated that translocated in liposarcoma (TLS), also termed FUS, is an interacting molecule of the p65 (RelA) subunit of the transcription factor nuclear factor kappaB (NF-kappaB) using a yeast two-hybrid screen. | | Human | PRPH | 5630 | peripherin | No peripherin expression was noted in any of eleven epithelioid sarcomas, eight liposarcomas, seven conventional chondrosarcomas, four neurothekeomas, three alveolar soft part sarcomas, or three clear cell sarcomas. | | Human | PPYR1 | 5540 | | The expression of the three catalytic subunits of protein phosphatase (PP) type 1 and 2A, PP1 alpha, PP1 gamma 1, and PP2AC, was examined in 8 cases of lipoma as a benign tumor and 4 cases of liposarcoma as a malignant tumor using immunohistochemical analysis. | | Human | PPP2CA | 5515 | protein phosphatase 2, catalytic subunit, alpha isozyme | The expression of the three catalytic subunits of protein phosphatase (PP) type 1 and 2A, PP1 alpha, PP1 gamma 1, and PP2AC, was examined in 8 cases of lipoma as a benign tumor and 4 cases of liposarcoma as a malignant tumor using immunohistochemical analysis. | | Human | NAB2 | 4665 | NGFI-A binding protein 2 (EGR1 binding protein 2) | The human NAB2 gene has been localized to chromosome 12ql3.3-14.1, a region that is rearranged in several solid tumors, lipomas, uterine leiomyomata, and liposarcomas. | | Human | MYF5 | 4617 | myogenic factor 5 | INFERRED, Score=800, UMLKSK CUI: C0023827 | | Human | MDM2 | 4193 | MDM2 oncogene, E3 ubiquitin protein ligase | High-level amplification of MDM2 at 12q13-15 was observed in 4/7 cases of liposarcoma Nutlin efficiently stabilized p53 and induced downstream p53 dependent transcription and apoptosis in liposarcoma cells with amplified MDM2 in vitro | | Human | MCF2 | 4168 | MCF.2 cell line derived transforming sequence | In addition, following transfection of DNA from a liposarcoma, we identified an activated gene that failed to hybridize to probes prepared from 10 known human oncogenes (K-ras, H-ras, N-ras, ret, met, trk, mas, dbl, raf and hst) that have previously been detected in DNA transfection experiments. |
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