PMID- 9482408 OWN - NLM STAT- MEDLINE DCOM- 19980305 LR - 20190914 IS - 1077-4114 (Print) IS - 1077-4114 (Linking) VI - 20 IP - 1 DP - 1998 Jan-Feb TI - Expression of the c-Myc protein in childhood medulloblastoma. PG - 18-25 AB - PURPOSE: The purpose of this study was to determine the incidence of c-Myc protein expression in medulloblastoma/primitive neuroectodermal tumor (MB/PNET) and to identify mechanisms in addition to c-myc gene amplification that lead to increased protein expression. METHODS: We analyzed c-myc gene copy number, mRNA level and protein expression in a panel of MB/PNET cell lines. C-Myc protein levels were assessed in tumor specimens and cell lines using immunohistochemical staining with a c-Myc-specific monoclonal antibody. RESULTS: Southern analysis confirmed c-myc gene amplification in the D425 MED cell line and re-arrangement of one allele in D283 MED, which was analyzed further and appeared to represent a small deletion 3' of exon 3. C-myc transcript levels were dramatically elevated in both lines. Using a c-myc probe, fluorescence in situ hybridization (FISH) showed c-myc present in 3 tandem copies at 8q24 in D283 MED and multiple copies as double minutes in D425 MED. Immunohistochemistry showed c-Myc protein expression in 9 of 10 tumors and all cell lines, regardless of gene amplification status or level of mRNA expression. CONCLUSIONS: c-Myc protein expression is common in MB/PNET tumor specimens and cell lines. Elevated protein levels are observed in the absence of amplification, suggesting that multiple mechanisms of c-myc dysregulation may be involved in MB/PNET. These studies support a role for c-Myc in the development of this common childhood tumor. FAU - Bruggers, C S AU - Bruggers CS AD - Department of Pediatrics, Center for Children at the Huntsman Cancer Institute, University of Utah School of Medicine, Salt Lake City 84112, USA. FAU - Tai, K F AU - Tai KF FAU - Murdock, T AU - Murdock T FAU - Sivak, L AU - Sivak L FAU - Le, K AU - Le K FAU - Perkins, S L AU - Perkins SL FAU - Coffin, C M AU - Coffin CM FAU - Carroll, W L AU - Carroll WL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Pediatr Hematol Oncol JT - Journal of pediatric hematology/oncology JID - 9505928 RN - 0 (Proto-Oncogene Proteins c-myc) RN - 0 (RNA, Messenger) SB - IM MH - Child MH - Child, Preschool MH - Genes, myc MH - Humans MH - Medulloblastoma/*chemistry/genetics MH - Proto-Oncogene Proteins c-myc/*analysis MH - RNA, Messenger/analysis MH - Tumor Cells, Cultured EDAT- 1998/03/03 00:00 MHDA- 1998/03/03 00:01 CRDT- 1998/03/03 00:00 PHST- 1998/03/03 00:00 [pubmed] PHST- 1998/03/03 00:01 [medline] PHST- 1998/03/03 00:00 [entrez] AID - 10.1097/00043426-199801000-00003 [doi] PST - ppublish SO - J Pediatr Hematol Oncol. 1998 Jan-Feb;20(1):18-25. doi: 10.1097/00043426-199801000-00003.