PMID- 7815081
OWN - NLM
STAT- MEDLINE
DCOM- 19950208
LR  - 20190512
IS  - 0022-3069 (Print)
IS  - 0022-3069 (Linking)
VI  - 54
IP  - 1
DP  - 1995 Jan
TI  - Alterations at chromosome 17 loci in peripheral nerve sheath tumors.
PG  - 65-73
AB  - Little is known about the molecular genetic changes in malignant peripheral nerve 
      sheath tumors (MPNST). Inactivation of the TP53 gene in 17p has been reported in 
      a few tumors. The MPNST is one of the manifestations of neurofibromatosis 1 
      (NF1), suggesting that the NF1 gene in 17q might be important. We present a study 
      of 15 neurofibromas and MPNST from nine individuals. Seven patients had NF1, and 
      six of these developed MPNST. Genetic alterations at nine polymorphic loci on 
      chromosome 17 were examined. Allelic imbalance was detected only in the malignant 
      tumors from NF1 patients (4/6). Complete loss of heterozygosity of 17q loci was 
      found in three of these tumors, all including loci within the NF1 gene. Two of 
      the malignant tumors also showed deletions on 17p. No mutations were detected 
      within exon 5-8 of the TP53 in any of the MPNST, and none of them were TP53 
      protein-positive using immunostaining with mono- and polyclonal antibodies 
      against TP53. The numbers of chromosome 17 present in each tumor were evaluated 
      by use of fluorescence in situ hybridization (FISH) on interphase nuclei with a 
      centromere-specific probe. A deviation from the disomic status of chromosome 17 
      was observed in two of the MPNST from NF1 patients. These results support the 
      hypothesis of inactivation of both NF1 gene alleles during development of MPNST 
      in patients with NF1. In contrast to other reports, we did not find evidence for 
      a homozygous mutated condition of the TP53 gene in the same tumors. Finally, FISH 
      analysis was in accordance with the DNA analysis in the deduction of the numbers 
      of chromosome 17 in these tumors.
FAU - Lothe, R A
AU  - Lothe RA
AD  - Department of Genetics, Norwegian Radium Hospital, Oslo.
FAU - Slettan, A
AU  - Slettan A
FAU - Saeter, G
AU  - Saeter G
FAU - Brogger, A
AU  - Brogger A
FAU - Borresen, A L
AU  - Borresen AL
FAU - Nesland, J M
AU  - Nesland JM
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - J Neuropathol Exp Neurol
JT  - Journal of neuropathology and experimental neurology
JID - 2985192R
RN  - 0 (Tumor Suppressor Protein p53)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Alleles
MH  - *Chromosome Aberrations
MH  - *Chromosomes, Human, Pair 17
MH  - Female
MH  - Gene Rearrangement
MH  - Humans
MH  - Immunologic Techniques
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Mutation
MH  - Peripheral Nervous System Neoplasms/*genetics/metabolism
MH  - Tumor Suppressor Protein p53/genetics/metabolism
EDAT- 1995/01/01 00:00
MHDA- 1995/01/01 00:01
CRDT- 1995/01/01 00:00
PHST- 1995/01/01 00:00 [pubmed]
PHST- 1995/01/01 00:01 [medline]
PHST- 1995/01/01 00:00 [entrez]
AID - 10.1097/00005072-199501000-00008 [doi]
PST - ppublish
SO  - J Neuropathol Exp Neurol. 1995 Jan;54(1):65-73. doi: 
      10.1097/00005072-199501000-00008.