Entry
Name
Transcriptional misregulation in cancer - Mus musculus (house mouse)
Description
In tumor cells, genes encoding transcription factors (TFs) are often amplified, deleted, rearranged via chromosomal translocation and inversion, or subjected to point mutations that result in a gain- or loss-of- function. In hematopoietic cancers and solid tumors, the translocations and inversions increase or deregulate transcription of the oncogene. Recurrent chromosome translocations generate novel fusion oncoproteins, which are common in myeloid cancers and soft-tissue sarcomas. The fusion proteins have aberrant transcriptional function compared to their wild-type counterparts. These fusion transcription factors alter expression of target genes, and thereby result in a variety of altered cellular properties that contribute to the tumourigenic process.
Class
Human Diseases; Cancer: overview
BRITE hierarchy
Pathway map
mmu05202 Transcriptional misregulation in cancer
Ortholog table
Organism
Mus musculus (house mouse) [GN:
mmu ]
Gene
12394 Runx1; runt related transcription factor 1 [KO:K08367 ]
12981 Csf2; colony stimulating factor 2 (granulocyte-macrophage) [KO:K05427 ]
12395 Runx1t1; RUNX1 translocation partner 1 [KO:K10053 ]
20466 Sin3a; transcriptional regulator, SIN3A (yeast) [KO:K11644 ]
20185 Ncor1; nuclear receptor co-repressor 1 [KO:K04650 ]
12606 Cebpa; CCAAT/enhancer binding protein alpha [KO:K09055 ]
14129 Fcgr1; Fc receptor, IgG, high affinity I [KO:K06498 ]
18854 Pml; promyelocytic leukemia [KO:K10054 ] [EC:2.3.2.-]
110794 Cebpe; CCAAT/enhancer binding protein epsilon [KO:K10051 ]
12047 Bcl2a1d; B cell leukemia/lymphoma 2 related protein A1d [KO:K02162 ]
12044 Bcl2a1a; B cell leukemia/lymphoma 2 related protein A1a [KO:K02162 ]
12045 Bcl2a1b; B cell leukemia/lymphoma 2 related protein A1b [KO:K02162 ]
12046 Bcl2a1c; B cell leukemia/lymphoma 2 related protein A1c [KO:K02162 ]
235320 Zbtb16; zinc finger and BTB domain containing 16 [KO:K10055 ]
93735 Wnt16; wingless-type MMTV integration site family, member 16 [KO:K01558 ]
13219 Defa-rs10; defensin, alpha, related sequence 10 [KO:K05230 ]
13221 Defa-rs12; defensin, alpha, related sequence 12 [KO:K05230 ]
13222 Defa-rs2; defensin, alpha, related sequence 2 [KO:K05230 ]
13223 Defa-rs4; defensin, alpha,, related sequence 4 [KO:K05230 ]
64144 Mllt1; myeloid/lymphoid or mixed-lineage leukemia; translocated to, 1 [KO:K15187 ]
70122 Mllt3; myeloid/lymphoid or mixed-lineage leukemia; translocated to, 3 [KO:K15187 ]
12393 Runx2; runt related transcription factor 2 [KO:K09278 ]
16599 Klf3; Kruppel-like transcription factor 3 (basic) [KO:K15605 ]
108829 Jmjd1c; jumonji domain containing 1C [KO:K11449 ] [EC:1.14.11.-]
22290 Uty; ubiquitously transcribed tetratricopeptide repeat containing, Y-linked [KO:K11447 ] [EC:1.14.11.68 ]
109115 Supt3; SPT3, SAGA and STAGA complex component [KO:K11313 ]
12576 Cdkn1b; cyclin dependent kinase inhibitor 1B [KO:K06624 ]
12580 Cdkn2c; cyclin dependent kinase inhibitor 2C [KO:K06622 ]
53623 Gria3; glutamate receptor, ionotropic, AMPA3 (alpha 3) [KO:K05199 ]
15242 Hhex; hematopoietically expressed homeobox [KO:K08024 ]
19208 Ptcra; pre T cell antigen receptor alpha [KO:K06056 ]
11797 Birc2; baculoviral IAP repeat-containing 2 [KO:K16060 ]
11796 Birc3; baculoviral IAP repeat-containing 3 [KO:K16060 ]
22029 Traf1; TNF receptor-associated factor 1 [KO:K03172 ]
19016 Pparg; peroxisome proliferator activated receptor gamma [KO:K08530 ]
94315 Prcc; papillary renal cell carcinoma (translocation-associated) [KO:K13105 ]
12575 Cdkn1a; cyclin dependent kinase inhibitor 1A [KO:K06625 ]
21417 Zeb1; zinc finger E-box binding homeobox 1 [KO:K09299 ]
16178 Il1r2; interleukin 1 receptor, type II [KO:K04387 ]
20732 Spint1; serine protease inhibitor, Kunitz type 1 [KO:K15619 ]
212980 Slc45a3; solute carrier family 45, member 3 [KO:K15379 ]
13714 Elk4; ELK4, member of ETS oncogene family [KO:K04376 ]
18109 Mycn; v-myc avian myelocytomatosis viral related oncogene, neuroblastoma derived [KO:K09109 ]
22059 Trp53; transformation related protein 53 [KO:K04451 ]
12151 Bmi1; Bmi1 polycomb ring finger oncogene [KO:K11459 ]
20683 Sp1; trans-acting transcription factor 1 [KO:K04684 ]
22642 Zbtb17; zinc finger and BTB domain containing 17 [KO:K10500 ]
18053 Ngfr; nerve growth factor receptor (TNFR superfamily, member 16) [KO:K02583 ]
14030 Ewsr1; Ewing sarcoma breakpoint region 1 [KO:K13209 ]
16009 Igfbp3; insulin-like growth factor binding protein 3 [KO:K10138 ]
260298 Fev; FEV transcription factor, ETS family member [KO:K09437 ]
11908 Atf1; activating transcription factor 1 [KO:K09053 ]
100040260 Atf1-ps; activating transcription factor 1, pseudogene [KO:K09053 ]
11864 Arnt2; aryl hydrocarbon receptor nuclear translocator 2 [KO:K15589 ]
17342 Mitf; melanogenesis associated transcription factor [KO:K09455 ]
18590 Pdgfa; platelet derived growth factor, alpha [KO:K04359 ]
16185 Il2rb; interleukin 2 receptor, beta chain [KO:K05069 ]
18124 Nr4a3; nuclear receptor subfamily 4, group A, member 3 [KO:K08559 ]
70439 Taf15; TATA-box binding protein associated factor 15 [KO:K14651 ]
13198 Ddit3; DNA-damage inducible transcript 3 [KO:K04452 ]
12608 Cebpb; CCAAT/enhancer binding protein beta [KO:K10048 ]
80859 Nfkbiz; nuclear factor of kappa light polypeptide gene enhancer in B cells inhibitor, zeta [KO:K14242 ]
18033 Nfkb1; nuclear factor of kappa light polypeptide gene enhancer in B cells 1, p105 [KO:K02580 ]
19697 Rela; v-rel reticuloendotheliosis viral oncogene homolog A (avian) [KO:K04735 ]
269397 Ss18l1; SS18, nBAF chromatin remodeling complex subunit like 1 [KO:K15623 ]
268996 Ss18; SS18, subunit of BAF chromatin remodeling complex [KO:K15623 ]
56312 Nupr1; nuclear protein transcription regulator 1 [KO:K15626 ]
68938 Aspscr1; ASPSCR1 tether for SLC2A4, UBX domain containing [KO:K15627 ]
13197 Gadd45a; growth arrest and DNA-damage-inducible 45 alpha [KO:K04402 ]
17873 Gadd45b; growth arrest and DNA-damage-inducible 45 beta [KO:K04402 ]
23882 Gadd45g; growth arrest and DNA-damage-inducible 45 gamma [KO:K04402 ]
107986 Ddb2; damage specific DNA binding protein 2 [KO:K10140 ]
Reference
Authors
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Comprehensive genomic screens identify a role for PLZF-RARalpha as a positive regulator of cell proliferation via direct regulation of c-MYC.
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Oncogenic homeodomain transcription factor E2A-Pbx1 activates a novel WNT gene in pre-B acute lymphoblastoid leukemia.
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Zelent A, Greaves M, Enver T
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Role of the TEL-AML1 fusion gene in the molecular pathogenesis of childhood acute lymphoblastic leukaemia.
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Authors
Sawinska M, Ladon D
Title
Mechanism, detection and clinical significance of the reciprocal translocation t(12;21)(p12;q22) in the children suffering from acute lymphoblastic leukaemia.
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Authors
Guenther MG, Lawton LN, Rozovskaia T, Frampton GM, Levine SS, Volkert TL, Croce CM, Nakamura T, Canaani E, Young RA
Title
Aberrant chromatin at genes encoding stem cell regulators in human mixed-lineage leukemia.
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Reference
Authors
Xia ZB, Popovic R, Chen J, Theisler C, Stuart T, Santillan DA, Erfurth F, Diaz MO, Zeleznik-Le NJ
Title
The MLL fusion gene, MLL-AF4, regulates cyclin-dependent kinase inhibitor CDKN1B (p27kip1) expression.
Journal
Reference
Authors
Wang QF, Wu G, Mi S, He F, Wu J, Dong J, Luo RT, Mattison R, Kaberlein JJ, Prabhakar S, Ji H, Thirman MJ
Title
MLL fusion proteins preferentially regulate a subset of wild-type MLL target genes in the leukemic genome.
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Title
Gains of 2p involving the REL locus correlate with nuclear c-Rel protein accumulation in neoplastic cells of classical Hodgkin lymphoma.
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Title
Constitutive NF-kappaB maintains high expression of a characteristic gene network, including CD40, CD86, and a set of antiapoptotic genes in Hodgkin/Reed-Sternberg cells.
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Title
Overexpression of c-maf is a frequent oncogenic event in multiple myeloma that promotes proliferation and pathological interactions with bone marrow stroma.
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Title
The MMSET histone methyl transferase switches global histone methylation and alters gene expression in t(4;14) multiple myeloma cells.
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Title
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Title
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Title
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Medendorp K, van Groningen JJ, Vreede L, Hetterschijt L, Brugmans L, van den Hurk WH, van Kessel AG
Title
The renal cell carcinoma-associated oncogenic fusion protein PRCCTFE3 provokes p21 WAF1/CIP1-mediated cell cycle delay.
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Title
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Title
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Title
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Title
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Title
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Title
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Title
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Title
Fusion oncogenes in tumor development.
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Reference
Authors
Fukuma M, Okita H, Hata J, Umezawa A
Title
Upregulation of Id2, an oncogenic helix-loop-helix protein, is mediated by the chimeric EWS/ets protein in Ewing sarcoma.
Journal
Reference
Authors
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Title
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Title
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Journal
Reference
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Jedlicka P
Title
Ewing Sarcoma, an enigmatic malignancy of likely progenitor cell origin, driven by transcription factor oncogenic fusions.
Journal
Int J Clin Exp Pathol 3:338-47 (2010)
Reference
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Title
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Reference
Authors
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Title
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Reference
Authors
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Title
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Journal
Reference
Authors
Filion C, Motoi T, Olshen AB, Lae M, Emnett RJ, Gutmann DH, Perry A, Ladanyi M, Labelle Y
Title
The EWSR1/NR4A3 fusion protein of extraskeletal myxoid chondrosarcoma activates the PPARG nuclear receptor gene.
Journal
Reference
Authors
Kim S, Lee HJ, Jun HJ, Kim J
Title
The hTAF II 68-TEC fusion protein functions as a strong transcriptional activator.
Journal
Reference
Authors
Sjogren H, Meis-Kindblom J, Kindblom LG, Aman P, Stenman G
Title
Fusion of the EWS-related gene TAF2N to TEC in extraskeletal myxoid chondrosarcoma.
Journal
Cancer Res 59:5064-7 (1999)
Reference
Authors
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Title
Myxoid liposarcoma FUS-DDIT3 fusion oncogene induces C/EBP beta-mediated interleukin 6 expression.
Journal
Reference
Authors
Goransson M, Andersson MK, Forni C, Stahlberg A, Andersson C, Olofsson A, Mantovani R, Aman P
Title
The myxoid liposarcoma FUS-DDIT3 fusion oncoprotein deregulates NF-kappaB target genes by interaction with NFKBIZ.
Journal
Reference
Authors
Willems SM, Schrage YM, Bruijn IH, Szuhai K, Hogendoorn PC, Bovee JV
Title
Kinome profiling of myxoid liposarcoma reveals NF-kappaB-pathway kinase activity and casein kinase II inhibition as a potential treatment option.
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Reference
Authors
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Title
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Journal
Reference
Authors
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Title
Identification of target genes regulated by PAX3 and PAX3-FKHR in embryogenesis and alveolar rhabdomyosarcoma.
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Reference
Authors
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Title
The SYT-SSX fusion protein down-regulates the cell proliferation regulator COM1 in t(x;18) synovial sarcoma.
Journal
Reference
Authors
Jun HJ, Lee J, Lim do H, Park JO, Ahn G, Seo SW, Sung KS, Lim do H, Yoo KH, Choi YL
Title
Expression of MET in alveolar soft part sarcoma.
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Title
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Reference
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Title
Recent advances in p53 research and cancer treatment.
Journal
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