EGAGenomicsExome Sequencinghttps://ega-archive.org/studies/EGAS00001003031EGAEGA study EGAS00001003031Patient-derived neuroblastoma model system OHC-NB1EGArestrictedKEK1, Kek1, Kek-1, anatomical systems, HNA-2a, Neuroblastomas, Neuroblastoma, DmelCG12283, kek, HNA2A, Dmkek1, n(2)k07332., patient, l(2)01433, BEST:GM02380, Client, kekon, kek-1, Patient, SD01674, Clients, Kek, CT18186, NB, PRV-1, PRV1, GM02380, CG12283, uns, NB1, NB1 GPprotein translation, KEK1, Kek1, IPP2A2, Antemortem Diagnosis, Metastasis, Laboratory, Heterograft, Mus domesticus, Neuroblastoma., Dmkek1, mycna, mycnb, Neoplasm Metastases, CASP-14, neoplasm metastasis, TRANSPL HETEROL, Tumor, Diagnosis, House Mouse, cancer metastasis, 5730420M11Rik, Background, chromosome 1p deletion, Partial monosomy of the short arm of chromosome 1, diseases, medulla ossea, Cultural, Kek, symptoms, Line, diseases and disorders, Whole Transcriptome, Transcriptome Sequencing, PRV1, 1p monosomy, NB1 GP, Diploid Cells, SET, ethnicity, human disease, tumor cell, TAF-I, Complete Exome Sequencing, Whole Transcriptome Sequencing, kek, Tissue, Swiss Mice, Nmyc1, Partial monosomy of chromosome 1p, N-myc, Red Marrow, DmelCG4299, IGAAD, set, monosomy 1p, Xenotransplantations, DmelCG10574, XN-myc, loss of chromosome 1p, Red, Homo sapiens disease, Malignancies, stage, Diploid, Diagnose, Tumors, phapii, screening, protein anabolism, protein biosynthetic process, partial deletion of the short arm of chromosome type 1, Exome, CASP14, mouse, StF-IT-1, Diagnoses, Cultural Background, Benign, Postmortem, Screenings, Cultures, Examinations and Diagnoses, Mini-ICE, Diseases, protein formation, simple tissue, Postmortem Diagnosis, Heterologous Transplantations, Lines, Diagnoses and Examination, xN-myc1, Xenograft, Mus musculus, Postmortem Diagnoses, Complete Transcriptome, Xenotransplantation, HNA-2a, HLA-DR-associated protein II, Cultural Beliefs, Caspase-14 subunit p10, Neuroblastomas, Transplantation, DI-2, medulla of bone, mice, I-2Dm, Heterogeneity, Swiss Mouse, signs, Benign Neoplasms, Caspase-14 subunit p19, l(2)01433, Xenografts, CG4299, BEST:GM02380, MICE, Partial deletion of chromosome 1p, Malignant Neoplasms, domesticus, I-2PP1, disease, metastatic, TAF-IBETA, protein synthesis, Patient, Cells, Whole Exome Sequencing, Mouse, TAF-Ibeta, other neoplasm, Transcriptome Sequencings, i2pp2a, uns, Bone, NB1, 3.4.22.-, other disease, Complete Exome, Nmyc, Neoplasms, developmental stage, ODED, Benign Neoplasm, mini-ICE, Transplantations, Malignant, PHAPII, heterologous transplantation, deletion 1p, mycn, House, SD01674, Genetic heterogeneity, Marrow, NMYC, Mass, disease or disorder, Screening, Mus musculus domesticus, Yellow, Antemortem, nmyc, Mice, CG12283, Backgrounds, 1p deletion, n(2)k07332, del(1p), tumor cell migration, WES, Complete, anatomical systems, Exome Sequencings, Genetic, Genetic Heterogeneities, Diploids, Malignancy, Plastic, Swiss, Flexibility, ipp2a2, 1p only deleted, Heterografts, 2pp2a, metastasis, Cultural Relativisms, Diagnoses and Examinations, Nmuc1, Sequencing, CG10574, non-neoplastic, Neoplasias, MODED, Whole Exome, 2PP2A, tumour cell, Karyotypes, taf-ibeta, Clients, Whole, partial deletion of the short arm of chromosome 1, heterogeneity, partial monosomy of chromosome 1p, dSET, dSet, Customs, disorder, culture, bHLHe37, partial monosomy 1p, tumor metastasis, XNmyc, Heterologous, Cancer, Antemortem Diagnoses, findings, Malignant Neoplasm, Complete Exome Sequencings, Metastases, disorders, HNA2A, igaad, Cell Lines, adherent cell line, medical condition, Cultural Backgrounds, Client, Examination and Diagnoses, Cell, group, Metastase, Loss of Chromosome 1p, 1p Deletion, Exome Sequencing, kek-1, XENOTRANSPL, medulla ossium, Mus, I-2PP2A, Mass Screenings, c-nmyc, Dm I-2, I2PP2A, Neoplasm, condition, protein biosynthesis, NB, PRV-1, background, Yellow Marrow, Kek-1, Complete Transcriptome Sequencing, ensemble, Neuroblastoma, medullary bone, DmelCG12283, Heterogeneities, HETEROL TRANSPL, House Mice, patient, Cancers, introduction, Nmyc-1, Laboratory Mice, kekon, dSET/TAF-Ibeta, 2610030F17Rik, Diploid Cell, CT18186, Relativisms, partial deletion of chromosome 1p, Diploidies, GM02380, Relativism, Cultural Relativism, AA407739, Laboratory Mouse, partial monosomy of the short arm of chromosome 1, NeoplasiafalsePatient-derived neuroblastoma model system OHC-NB1Accurate disease modeling presents a bottleneck against effective translation of novel neuroblastoma therapies into the clinic. Recently, considerable intra-tumor genetic heterogeneity has been observed in neuroblastoma, which calls for preclinical models that reflect this feature. Flexibility in a testing platform is also desirable to support assessment of different endpoints and tumor cell characteristics. We present the novel neuroblastoma model OHC-NB1, propagated from a bone marrow metastasis from a patient with INRG stage M, MYCN-amplified neuroblastoma at first diagnosis. To model different aspects of the disease, several preclinical models were developed from the bone marrow aspirate: a tissue plastic-adherent cell line, a 3-dimensional (3D) spheroid culture, and cell line-derived xenografts that were subcutaneously passaged in mice. Typical aspects of MYCN-amplified neuroblastoma, such as MYCN amplification in double minutes, 1p deletion, 17q gain, and a diploid karyotype, are present in OHC-NB1 and persist in all culture types. Whole exome sequencing revealed intra-tumor heterogeneity in the bone marrow metastasis reflected in the culture models. The set of OHC-NB1 cultures with a genetically heterogeneous MYCN-amplified neuroblastoma background provide a flexible preclinical testing system that recapitulates important features of metastatic neuroblastoma.2019-08-05 15:53:59EGAS000010030319606EGAD00001004138EGAC00001000923