Localization of a hereditary neuroblastoma predisposition gene to 16p12-p13.
BACKGROUND
Hereditary predisposition to develop neuroblastoma segregates as an autosomal dominant Mendelian trait.


METHODS
We have performed linkage analysis on 10 families with neuroblastoma to localize a hereditary neuroblastoma predisposition gene (HNB1).


RESULTS
A single genomic interval at chromosome bands 16p12-p13 was consistent with linkage (lod = 3.46), and identification of informative recombinants defined a 25.9-cM critical region between D16S748 and D16S3068. Loss of heterozygosity was identified in 5/12 familial (42%) and 55/259 nonfamilial (21%) neuroblastomas at multiple 16p polymorphic loci. A 12.8-cM smallest region of overlap of deletions was identified within the interval defined by linkage analysis (tel-D16S764-D16S412-cen).


CONCLUSIONS
Taken together, these data suggest that HNB1 is located at 16p12-p13 and that inactivation of this gene may contribute to the pathogenesis of nonfamilial neuroblastomas.
Weiss
M J
MJ
Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4318, USA.
Guo
C
C
Shusterman
S
S
Hii
G
G
Mirensky
T L
TL
White
P S
PS
Hogarty
M D
MD
Rebbeck
T R
TR
Teare
D
D
Urbanek
M
M
Brodeur
G M
GM
Maris
J M
JM
D002648:Child; D002872:Chromosome Deletion; D002874:Chromosome Mapping; D002885:Chromosomes, Human, Pair 16 / Q000235:genetics*; D008040:Genetic Linkage; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006801:Humans; D019656:Loss of Heterozygosity; D009447:Neuroblastoma / Q000235:genetics*; D010375:Pedigree