[CANCER RESEARCH 54, 6353-6358, December 15, 19941
Advances in Brief
Deletion ofpl6 and pl5 Genes in Brain Tumors1
Jin Jen, J. Wade Harper, Sandra H. Bigner, Darell D. Bigner, Nickolas Papadopoulos, Sanford Markowitz,
James K. V. Wilson, Kenneth W. Kinzler, and Bert Vogelstein2
The Johns Hopkins Oncology Center, Baltimore, Maryland 21231 Ii. J., N. P., K. W. K., B. V.]; the Verna and Marrs McLean Department of Biochemistry, Baylor College of
Medicine, Houston, Texas 77030 Ii. W. H.]; the Department of Pathology. Duke University School of Medicine, Durham, North Carolina 27710 (5. H. B., D. D. B.]; and the
Department of Medicine and Ireland Cancer Center, University Hospital of Cleveland and Case Western Reserve University, Cleveland, Ohio 44106 (S. M.. J. K. V. W.]
Abstract
We have used molecular genetic methods to examine the status of cell
cycle-inhibitory genes in human brain tumors. We found that p16 and a
neighboring gene, pi5, were often homozygously deleted in glioblastoma
multiformes but not in medulloblastomas or ependymomas. The deletions
occurred in both primary tumors and their derived xenografts, but no
Intragenic mutations In either of the two genes were found. TheplS gene
was expressed in a more widespread pattern in normal tissues than pl6,
but the products of both genes had similar capacities to bind to cydlin
D-dependent kinases 4 and 6. These data suggest that the target of deletion
in glioblastoma multiforme includes both p15 and p16 genes. The reason
that homozygous deletions, rather than intragenic mutations, are so corn
mon in these tumors rnay be that deletion is a more efficient mechanism
for simultaneous inactivation of both genes.
Introduction
The Cdks3 are essential enzymes with activities that are tightly
regulated by protein phosphorylation and association with other pro
teins, particularly the cyclins (i, 2). Sequentiai activation of the cy
ciin-Cdk complexes is thought to be responsible for orderly transitions
through the cell cycle. The abnormal activation of Cdk activity,
through a variety of mechanisms, is expected to underlie part of the
uncontrolled growth that characterizes cancer.
Two classes of genes whose products can inhibit Cdks have re
cently been identified. The first class consists of p21 [also called
dpi, WAFJ, SDIi,CAP2O (3—8)] andp27[alsocalledKIPi,(9—il)]
which have a high degree of sequence similarity and can inhibit a
variety of Cdk subtypes. The expression of p2i is regulated by p53,
providing a link between tumor suppressor genes and cell cycle
regulation (5). The second class is represented by pitS (also called
MTSJ), which encodes a protein with ankyrin-like repeats that can
inhibit Cdk4 and Cdk6 but not other Cdks (12, i3). Cdk4 and Cdk6
are closely related in primary sequence and are both activated by D
type cyclins during G1 (14, 15).
Previous studies have demonstrated that many tumors, particularly
melanomas, leukemias, and gliomas, contain hemizygous or homozy
gous deletions of chromosome 9p2i (16—18). Additionally, a gene
responsible for melanoma predisposition has been mapped to this
same chromosomai region by linkage analysis (19). The pitS gene
Received 10/11/94; accepted 11/3/94.
The costs of publication of this article were defrayed in part by the payment of page
charges. This articie must therefore be hereby marked advertisement in accordance with
18 U.S.C. Section 1734 solely to indicate this fact.
1 This work was supported by the Preuss Foundation; the Clayton Fund; the National
Foundation for Cancer Research; Baylor SPORE in Prostate Cancer; NIH Grants CA
58204 (J. W. H.), AG-i1085 (S. J. E., J. W. H.), CA-43733 and CA-51504 (S. H. B.),
CA-i1898 and NS-20023 (D. D. B.), CA-57208 (S. M.), CA-51183 (J. W.), CA-4i183
(B.V.),andCA-09243(J.J.).B.V.is anAmericanCancerSocietyResearchProfessor.
2 To whom requests for reprints should be addressed.
3 The abbreviations used are: Cdk, cyclin-dependent kinase; MTS, multiple tumor
suppressor gene; CIP1, Cdk-interacting protein; WAF1, wild-type p53-activated
fragment; SDI1, senescent cell-derived inhibitor; CAP1, Cdk-associated protein; KIP1,
Cdk-inhibitory protein; RT, reverse transcriptase; GM, glioblastoma multiforme; ORF,
open reading frame.
resides within the deleted 9p2i region within these tumors (20, 2i).
Subtle mutations of the pi6 gene have been reported in a variety of
tumor cell lines and in some primary tumors (20—23). Moreover,
germline alterations of the pitS gene have been found in a subset of
patients with familial melanoma (21, 24, 25). There has been consid
erabie controversy over the role of pitS in neopiasia, in part because
of conflicting results that have been reported (20—29).
In this study, we have searched for genetic aiterations of p16 in
human brain tumors. During the course of this work, we were led to
characterize a neighboring gene, p15, that proved to have biochemical
properties similar to those of pi6. Our results suggest that both of
these genes may play a role in gliobiastoma tumorigenesis.
Materials and Methods
Tumor and Nucleic Acid Sources. Brainand colorectaltumorxenografts
were established in nude mice as described previously (30). DNA from
primary and xenografted tumors were prepared by standard methods (31).
RNA from normal human adult tissues and GM xenografts were purified by a
guanidine isothiocyanate based method (Promega) following the manufactur
er's instructions. Placenta and HeLa cell polyadenylated mRNA were pur
chased from Stratagene (San Diego, CA).
PCR Amplification and Sequencing of Tumor DNA. Primersused to
amplify pi6 exons are listed in Table 1. All PCR reactions were carried out in
50-pA reactions using 100 ng genomic DNA. The PCR solutions have been
described (32), and the reaction used a 95°C,2-mm initial denaturation step
followed by 30 to 35 cycles at 95°C for 30 s, 58°C for 1 mm, and 70°C for 1
min. The PCR products were separated by electrophoresis on 1.5% agarose or
10% nondenaturing polyacrylamide gels. Mouse spleen DNA was used to
ensure that the PCR primers were specific for human pitS. Sequencing of the
PCR products was performed with Sequimerm Polymerase (Epicentre) as
described by the manufacturer, using the primers listed in Table 1. Amplifi
cation of pi5 was carried out similarly, using the primers described in Table
1. A 500-base pair genomic Cdk4 fragment(33) was amplified in parallel PCR
experiments to control for the integrity of DNA samples.
p's cDNACloning. The5'endofthepi5cDNAwasobtained through the
rapid amplification of cDNA ends procedure, using placenta cDNA as template
(34, 35). The rapid amplification of cDNA ends anchor primer (Clontech) was
combined with either primer P15-2R or primer P15-54 (Table 1) for the first
and second rounds of PCR amplification, respectively. A PCR product of about
800 base pairs generated after the second PCR amplification was cloned
directly into the pCRII vector (Invitrogen). The sequences of the clones were
determined using 17 and SP6 primers as well as additional primers from the
p15 gene as its sequence was generated. The sequence was confirmed by direct
sequencing of a P1 clone (P2420) containing the entire pi5 and p16 genes
usingpi5-specific primers. The P2420 clone was isolated by PCR screening of
a human P1 genomic library (Genome Systems, Inc.)
Functional Analyses of p15. RT-PCR products containing the complete
coding sequence of the pi6 and pi5 genes were generated from HeLa and
placenta mRNA, respectively, using primers containing signals for in vitro
transcription by 11 polymerase and in vitro translation by reticulocyte lysates
(Table 1, primers TNT-Pi6 and P16-S13 for pi6 and primers TNT-P15 and
P15-S2R for pi5). The p16 and p15 proteins for in vitro binding assays were
synthesized using these RT-PCR products and an in vitro transcription-trans
lation kit (Promega) as described previously for other genes (36, 37).
6353
Research.
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