PMID- 8993980
OWN - NLM
STAT- MEDLINE
DCOM- 19970310
LR  - 20191024
IS  - 1045-2257 (Print)
IS  - 1045-2257 (Linking)
VI  - 18
IP  - 1
DP  - 1997 Jan
TI  - High-resolution deletion mapping of chromosome arm 17p in childhood primitive 
      neuroectodermal tumors reveals a common chromosomal disruption within the 
      Smith-Magenis region, an unstable region in chromosome band 17p11.2.
PG  - 50-8
AB  - Loss of heterozygosity (LOH) on chromosome arm 17p is the most common genetic 
      aberration in childhood primitive neuroectodermal tumors (PNETs). To determine 
      the frequency and extent of 17p deletions, 29 loci on 17p were investigated in 24 
      tumors by using restriction fragment length polymorphism (RFLP) and 
      microsatellite analysis. LOH on 17p was found in 9 of 24 tumors. In all tumors 
      with LOH, a continuous stretch from the telomere to chromosome band 17p11.2 was 
      completely deleted, and no interstitial or terminal small-scale deletions were 
      detected in the remaining 15 tumors. In four tumors with LOH on 17p, the 
      chromosomal breakpoint was located between D17S953 and D17S805. To identify this 
      deletion breakpoint on the cytogenetic map of chromosome 17 and to exclude 
      uniparental disomy, we verified our data by using fluorescence in situ 
      hybridization (FISH) analyses. By using two yeast artificial chromosome (YAC) 
      clones that were positive for D17S689 and D17S953, the same breakpoint was 
      confirmed in two specimens of cerebrospinal fluid (CSF) metastases by using FISH 
      on interphase preparations. We demonstrate that, in most childhood PNETs with LOH 
      on 17p, the breakpoint is close to, but not within, the centromere. It varies, 
      and it occurs predominantly between the two markers D17S689 and D17S953, which is 
      an unstable chromosomal region that is deleted or duplicated in the Smith-Magenis 
      syndrome. Because LOH of 17p is associated with the formation of isochromosome 
      17q in the majority of PNETs, this study provides entry points to determine the 
      molecular nature of this phenomenon.
FAU - Scheurlen, W G
AU  - Scheurlen WG
AD  - Department of Pediatrics, University of Wurzburg, Germany.
FAU - Seranski, P
AU  - Seranski P
FAU - Mincheva, A
AU  - Mincheva A
FAU - Kuhl, J
AU  - Kuhl J
FAU - Sorensen, N
AU  - Sorensen N
FAU - Krauss, J
AU  - Krauss J
FAU - Lichter, P
AU  - Lichter P
FAU - Poustka, A
AU  - Poustka A
FAU - Wilgenbus, K K
AU  - Wilgenbus KK
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Genes Chromosomes Cancer
JT  - Genes, chromosomes & cancer
JID - 9007329
RN  - 0 (DNA, Neoplasm)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Brain Neoplasms/cerebrospinal fluid/*genetics
MH  - Child
MH  - Child, Preschool
MH  - *Chromosome Aberrations
MH  - Chromosome Deletion
MH  - *Chromosome Mapping
MH  - *Chromosomes, Human, Pair 17
MH  - DNA, Neoplasm/analysis
MH  - Female
MH  - Gene Deletion
MH  - Genes, Tumor Suppressor
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Interphase
MH  - Male
MH  - Metaphase
MH  - Microsatellite Repeats
MH  - Neuroectodermal Tumors/cerebrospinal fluid/*genetics
MH  - Polymorphism, Restriction Fragment Length
EDAT- 1997/01/01 00:00
MHDA- 2000/06/20 09:00
CRDT- 1997/01/01 00:00
PHST- 1997/01/01 00:00 [pubmed]
PHST- 2000/06/20 09:00 [medline]
PHST- 1997/01/01 00:00 [entrez]
AID - 10.1002/(SICI)1098-2264(199701)18:1<50::AID-GCC6>3.0.CO;2-0 [pii]
AID - 10.1002/(sici)1098-2264(199701)18:1<50::aid-gcc6>3.0.co;2-0 [doi]
PST - ppublish
SO  - Genes Chromosomes Cancer. 1997 Jan;18(1):50-8. doi: 
      10.1002/(sici)1098-2264(199701)18:1<50::aid-gcc6>3.0.co;2-0.