Table 1.

Fold of induction was normalized against that of the vector reporter without an activator

(A) Selected genes with PAX3-FKHR binding sites
IDEntrez gene nameLocationTypeDrugs
Self-renewal, growth, survival, oncogenesis
ALKAnaplastic lymphoma receptor tyrosine kinasePlasma membraneReceptor kinasePF-02341066
FGFR2Fibroblast growth factor receptor 2Plasma membraneReceptor kinaseTKI-258, AZD2171, brivanib, PHA-739358
FGFR4Fibroblast growth factor receptor 4Plasma membraneReceptor kinase
IGF1RInsulin-like growth factor 1 receptorPlasma membraneReceptor kinaseCP-751871, AMG479, R1507, MK-0646, AVE1642
KDRKinase insert domain receptor, VEGFR2Plasma membraneReceptor kinaseBevacizumab, sunitinib, vatalanib, sorafenib, vandetanib
MEIS1Meis homeobox 1NucleusTranscription factor
MYCNv-myc myelocytomatosis viral related oncogene, neuroblastoma-derivedNucleusTranscription factor
SPRY1Sprouty homologue 1, antagonist of FGF signaling (Drosophila)Plasma membraneFGFR4 ligand
Migration, metastasis
CXCR4Chemokine (C-X-C motif) receptor 4Plasma membraneGPCRJM 3100
METmet proto-oncogene (hepatocyte growth factor receptor)Plasma membraneReceptor kinasePF-02341066
Myogenic differentiation
EYA2Eyes absent homologue 2 (Drosophila)NucleusPhosphatase
EYA4Eyes absent homologue 4 (Drosophila)CytoplasmPhosphatase
MEOX1Mesenchyme homeobox 1NucleusTranscription factor
MEOX2Mesenchyme homeobox 2NucleusTranscription factor
MYF5/6Myogenic factor 6 (herculin)NucleusTranscription factor
MYOD1Myogenic differentiation 1NucleusTranscription factor
PRRX1Paired related homeobox 1NucleusTranscription factor
Other potential targets
ABAT4-Aminobutyrate aminotransferaseCytoplasmEnzymeValproic acid
ADRA1DAdrenergic, α-1D-, receptorPlasma membraneGPCRPaliperidone, risperidone, antazoline, acetaminophen, epinephrine, acetaminophen
ADRA2AAdrenergic, α-2A-, receptorPlasma membrane
ADRA2CAdrenergic, α-2C-, receptorPlasma membrane
DHFRDihydrofolate reductaseUnknownEnzymePyrimethamine, trimethoprim, iclaprim, methotrexate
(B) Verification of PAX3-FKHR binding site via enhancer assay
GeneEnhancer no.Binding intensityFold induction by activatorsRel. LocationDist. Tx Start bpDist. Tx End bp
NonePAX3PAX3-FKHR
VectorNoneNA1.01.00.9NANANA
ALK1High2.610.819.53rd Intron−262,835464,601
FGFR41Low1.51.93.73′ downstream−8,913−839
FGFR42High1.95.78.33′ downstream−16,273−8,199
IGF1R1Med1.84.04.82nd Intron−136,375171,486
MET1Low3.910.27.43rd Intron−35,32961,711
MET2High1.32.53.818th Intron−83,27713,763
MYCN1Med0.93.85.43′ downstream−96,819−92,786
MEOX11Med1.22.45.45′ upstream99420,274
MEOX21High0.80.83.81 intron−79873,218
PRRX11Med0.62.118.21st intron−4,22667,742