Linked Life Data

16186793

Source:http://linkedlifedata.com/resource/pubmed/id/16186793

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
2006-2-16
pubmed:abstractText
Supratentorial primitive neuroectodermal tumors (sPNET) and atypical teratoid/rhabdoid tumors (AT/RT) of the CNS represent a biological and clinical enigma, despite advances in both molecular techniques and clinical management for these two rare embryonal brain tumors of childhood. Epigenetic changes hold great potential as possible disease mechanisms and may be manipulated therapeutically. We thus studied aberrant methylation of the genes RASSF1A and CASP8 and its consequence on expression in cell lines and primary tumors using a combination of semiquantitative methylation specific PCR (MSP), bisulfite sequencing and RT-PCR. In all, 17 samples of autopsy-derived normal appearing brain served as controls. Opposed to control tissues 19/24 sPNET and 4/6 AT/RT demonstrated aberrant methylation for the RASSF1A promoter region. Treatment of cell lines using 5-Aza-2'-deoxycytidine (5AZA) alone or in combination with trichostatin A (TSA) succeeded in re-establishing expression of RASSF1A in cell lines derived from a renal rhabdoid, an AT/RT and a medulloblastoma. A 5' CpG-rich region of CASP8 was methylated in normal tissues and in tumors. However, CASP8 showed inconsistent expression patterns in normal and tumor tissues. Our results indicate that aberrant methylation of the RASSF1A promoter region may be of importance in the origin and progression of sPNET and AT/RT while the analysed 5'-CpG rich region of the CASP8 gene does not seem to play an important role in these tumors. Further studies of epigenetic changes in these rare tumors are warranted as their biology remains obscure and treatment efforts have been rather unsuccessfull.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0950-9232
pubmed:author
pubmed:issnType
Print
pubmed:day
16
pubmed:volume
25
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1111-7
pubmed:dateRevised
2008-11-21
pubmed:meshHeading
pubmed-meshheading:16186793-Adolescent, pubmed-meshheading:16186793-Adult, pubmed-meshheading:16186793-Azacitidine, pubmed-meshheading:16186793-Brain Neoplasms, pubmed-meshheading:16186793-Caspase 8, pubmed-meshheading:16186793-Caspases, pubmed-meshheading:16186793-Child, pubmed-meshheading:16186793-Child, Preschool, pubmed-meshheading:16186793-CpG Islands, pubmed-meshheading:16186793-DNA Methylation, pubmed-meshheading:16186793-Epigenesis, Genetic, pubmed-meshheading:16186793-Female, pubmed-meshheading:16186793-Gene Silencing, pubmed-meshheading:16186793-Humans, pubmed-meshheading:16186793-Hydroxamic Acids, pubmed-meshheading:16186793-Infant, pubmed-meshheading:16186793-Male, pubmed-meshheading:16186793-Neuroectodermal Tumors, Primitive, pubmed-meshheading:16186793-Promoter Regions, Genetic, pubmed-meshheading:16186793-Rhabdoid Tumor, pubmed-meshheading:16186793-Teratoma, pubmed-meshheading:16186793-Tumor Suppressor Proteins
pubmed:year
2006
pubmed:articleTitle
Epigenetic repression of RASSF1A but not CASP8 in supratentorial PNET (sPNET) and atypical teratoid/rhabdoid tumors (AT/RT) of childhood.
pubmed:affiliation
Department of Pediatric Hematology and Oncology, University Children's Hospital Muenster, Germany.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't