EGAGenomicsN/Ahttps://ega-archive.org/datasets/EGAD000010054359EGA dataset EGAD00001005435EGATERT promoter mutations reactivate telomerase, allowing for indefinite telomere maintenance and enabling cellular immortalization. These mutations specifically recruit the multimeric ETS factor GABP, which can form two functionally independent transcription factor species: a dimer or a tetramer. We show that genetic disruption of GABPβ1L (β1L), a tetramer-forming isoform of GABP that is dispensable for normal development, results in TERT silencing in a TERT promoter mutation-dependent manner. Reducing TERT expression by disrupting β1L culminates in telomere loss and cell death exclusively in TERT promoter mutant cells. Orthotopic xenografting of β1L-reduced, TERT promoter mutant glioblastoma cells rendered lower tumor burden and longer overall survival in mice. These results highlight the critical role of GABPβ1L in enabling immortality in TERT promoter mutant glioblastoma.Disruption of the β1L Isoform of GABP Reverses Glioblastoma Replicative Immortality in a TERT Promoter Mutation-Dependent Manner.Mancini Andrew A, Xavier-Magalhães Ana A, Woods Wendy S WS, Nguyen Kien-Thiet KT, Amen Alexandra M AM, Hayes Josie L JL, Fellmann Christof C, Gapinske Michael M, McKinney Andrew M AM, Hong Chibo C, Jones Lindsey E LE, Walsh Kyle M KM, Bell Robert J A RJA, Doudna Jennifer A JA, Costa Bruno M BM, Song Jun S JS, Perez-Pinera Pablo P, Costello Joseph F JFData Set.Desc, Description, Descriptive, Provide., Supplied, Supply, Descriptor, description, Provided, Product Description/Appearance, DESCRglioblastoma multiforme, grade IV adult astrocytic tumour, Glioblastoma, grade IV adult astrocytic tumor, TCS1, spongioblastoma multiforme, DKCA2, Glioblastomas, GBM, TRT, Giant Cell, Grade IV, Giant Cell Glioblastoma, Astrocytomas, Astrocytoma, hTRT, adult glioblastoma multiforme, Glioblastoma Multiforme, Grade IV Astrocytomas, CMM9, PFBMFT1, grade IV adult Astrocytic tumor, primary glioblastoma multiforme, EST2, hEST2, DKCB4, glioblastoma, Mutations., Grade IV Astrocytoma, TP2, Giant Cell Glioblastomas, TRForms, 3.4.22.-, T-cell leukemia, telomeric region, telomere, single-organism developmental process, Burden, Laboratory, Tumor Weight, postnatal development, Mus domesticus, AGL4, mini-ICE, growth and development, CASP-14, SEPALLATA 2, Grade IV, Tumor, Transcription Factor, Astrocytomas, House Mouse, hTRT, Tumor Weights, Mutations, Load, zinc ion regulated core promoter proximal region sequence-specific DNA binding, reduced, House, Roles, Telomerase Catalytic, RNA polymerase II distal enhancer sequence-specific DNA binding transcription factor activity, hEST2, DKCB4, nuclear chromosome, Mus musculus domesticus, Concepts, glioblastoma, Tumor Load, Volume, tiny, TP2, Giant Cell Glioblastomas, Mice, average, Transcription, telomeres, grade IV adult astrocytic tumor, DKCA2, Subunit, Swiss, Loads, hypoplasia, F10N7_150, Weights, Swiss Mice, Giant Cell Glioblastoma, RNA polymerase II core promoter proximal region sequence-specific DNA binding transcription factor activity, Astrocytoma, genetic, Glioblastoma Multiforme, Reverse Transcriptase, Catalytic Subunit, CMM9, RNA polymerase II proximal promoter sequence-specific DNA binding, Telomerase Reverse Transcriptase Catalytic Subunit, grade IV adult Astrocytic tumor, Telomerase Catalytic Subunit, copper ion regulated proximal promoter sequence-specific DNA binding, accidental cell death, Role Concepts, AGAMOUS-like 4, zinc ion regulated proximal promoter sequence-specific DNA binding, sequence-specific transcription regulatory region DNA binding RNA polymerase II transcription factor recruiting transcription factor activity, species, necrosis, Grade IV Astrocytoma, Tumor Volume, INSDC_feature:telomere, Grade IV., constitutitional genetic, Tumor Loads, TRANSCRIPTION FACTOR, TR, RNA polymerase II distal enhancer sequence-specific binding, small, RNA polymerase II core promoter proximal region sequence-specific binding, Telomerase Reverse Transcriptase, grade IV adult astrocytic tumour, familial, CASP14, mouse, Telomerase Reverse, zinc ion regulated core promoter proximal region sequence-specific DNA binding RNA polymerase II transcription factor activity, homeobox 1, TRT, Factor, Giant Cell, metal ion regulated sequence-specific DNA binding RNA polymerase II transcription factor activity, telomeric sequence, Telomerase, results, Cell, Concept, transcription factor activity, development, adult glioblastoma multiforme, Role Concept, Mus, EST2, primary glioblastoma multiforme, Weight, Mini-ICE, Role, RNA polymerase II transcription factor activity, Telomeres, sequence-specific distal enhancer binding RNA polymerase II transcription factor activity, Death, metal ion regulated core promoter proximal region sequence-specific DNA binding RNA polymerase II transcription factor activity, KNOTTED1-like homeobox gene 5, regulation of telomere length, F14P3.4, glioblastoma multiforme, Mus musculus, Factors, Glioblastoma, Caspase-14 subunit p10, TCS1, underdeveloped, spongioblastoma multiforme, mice, postnatal growth, Swiss Mouse, F14P3_4, Glioblastomas, GBM, House Mice, Caspase-14 subunit p19, metal ion regulated core promoter proximal region sequence-specific binding, copper ion regulated core promoter proximal region sequence-specific binding, MICE, Transcription factor, F10N7.150, Laboratory Mice, domesticus, Grade IV Astrocytomas, PFBMFT1, Transcriptase, telomeric DNA, copper ion regulated core promoter proximal region sequence-specific DNA binding RNA polymerase II transcription factor activity, inherited genetic, Mouse, metal ion regulated sequence-specific DNA binding, hereditary, growth, Laboratory Mouse, metal ion regulated proximal promoter sequence-specific DNA bindingfalseJ.Costello_20191016_dataset_rnaseq - samplesDescription not provided2019-11-13 09:51:36EGAD00001005435960630205050EGAC00001000153EGAS00001002582