[CANCER RESEARCH 64, 538 –546, January 15, 2004]
E6 and E7 Oncoproteins Induce Distinct Patterns of Chromosomal Aneuploidy in
Skin Tumors from Transgenic Mice
Anthony J. Schaeffer,
1
Marie Nguyen,
2
Amy Liem,
2
Denis Lee,
2
Cristina Montagna,
1
Paul F. Lambert,
2
Thomas Ried,
1
and Michael J. Difilippantonio
1
1
Genetics Branch, Center for Cancer Research, National Cancer Institute/NIH, Bethesda, Maryland; and
2
McArdle Laboratory for Cancer Research, University of Wisconsin
Medical School, Madison, Wisconsin
ABSTRACT
Inactivation of the tumor suppressor genes p53 and Rb are two of the
most common genetic alterations in cancer cells. We use a mouse model to
dissect the consequences of compromising the function of either of these
genes on the maintenance of genomic stability. Thirteen cell lines estab-
lished from skin tumors of mice expressing either the E6 or E7 oncopro-
tein of the human papillomavirus (HPV) type 16 under control of the
keratin 14 promoter were analyzed by comparative genomic hybridiza-
tion, spectral karyotyping and fluorescence in situ hybridization, reverse
transcription-PCR, and mutation analysis. Deducing from the wealth of
molecular cytogenetic data available from human cancers, we hypothe-
sized that the more benign tumors in mice expressing E7 would be distinct
from the more aggressive lesions in E6 transgenic mice. Tumorigenesis in
E6-expressing mice required specifically the selection and maintenance of
cells with extra copies of chromosome 6. Aneuploidy of chromosome 6 was
independent of activating mutations in H-ras on chromosome 7. Expres-
sion of either E6 or E7 resulted in centrosome aberrations, indicating that
each viral oncoprotein interferes independently with the centrosome cycle.
Although centrosome aberrations are consistent with development of
aneuploidy, no direct correlation was evident between the degree of
aneuploidy and the percentage of cells with aberrant centrosomes. Our
results show that although aneuploidy and centrosome aberrations are
present in tumor cells from mice expressing either E6 or E7, tumorigenesis
via E6 requires copy number increases of mouse chromosome 6, which is
partially orthologous to human chromosome 3q, a region gained in HPV-
associated carcinomas.
INTRODUCTION
The retinoblastoma susceptibility gene (Rb) and p53 are tumor
suppressor genes whose proper functioning is vital to maintaining
genomic stability and normal cell growth and differentiation. Re-
sponding to extracellular and intracellular signals, Rb is responsible
for the correct timing of the G1-S transition and for regulating the S,
G2, and M phases of the cell cycle. Inactivation of Rb leads to
genomic instability by impaired growth control, imprecise timing of
DNA synthesis, and chromosome mis-segregation (1). As an integral
member of the DNA damage control pathway, p53 functions in the
recognition and repair of damaged DNA and in programmed cell
death. The loss of wild-type p53 eliminates a major roadblock in
tumorigenesis, allowing cells with damaged DNA to proceed through
the cell cycle, thereby propagating genomically unstable progeny
containing random mutations, gene amplifications, chromosomal re-
arrangements, and/or aneuploidy (2).
Unfaithful chromosome segregation during mitosis can lead to
aneuploidy, a genetic defect observed consistently in tumor cells.
Proper chromosome segregation depends on attachment of mitotic
spindles to the kinetochore of each chromosome. The centrosome,
responsible for nucleating and organizing mitotic spindles, is an
essential component of this process (3). As such, the centrosome
duplication cycle is synchronized with the cell division cycle, and
uncoupling these cycles results in centrosome numbers above the
normal 1–2 per cell. Increased centrosome numbers can lead to
multipolar mitoses, mis-segregation of chromosomes, and genomic
instability (4). Numerical and/or structural centrosome abnormalities
have been reported in a variety of cancers, including breast cancer (5),
colon cancer (6), pancreatic cancer (7), head and neck cancer (8), and
human papillomavirus (HPV)-associated squamous cell carcinoma
(9), and is observed in a variety of mouse models of human cancers
(10 –12).
The respective roles that p53 and Rb play in centrosome duplication
are unclear. Duensing et al. (9) found that cells expressing a mutant
E7 protein unable to inactivate Rb had threefold-less centrosome
amplification than cells expressing an Rb-inactivating E7 protein. The
E7 mutant was equivalent roughly with respect to centrosome ampli-
fication to the untransfected control, and thus their results linked Rb
functionally to proper centrosome duplication. This is in contrast to
other studies in which the absence or inhibition of Rb function had
either no affect on centrosome number (8, 13) or depended on the
mode of Rb inactivation (14). Likewise, there also has been debate as
to whether centrosomes are affected by the absence of p53 function.
Carroll et al. (8) and Fukusawa et al. (13) have shown that p53
deficiency induces directly increased centrosome numbers. In con-
trast, centrosome amplification was observed in epidermal tumors
from mice expressing a mutant form of p53 but not in p53 knockout
mice after treatment with 7,12-dimethylbenz(a)anthracene (DMBA)
and 12-O-tetradecanoyl-phorbol-13-acetate (TPA) (15, 16). Thus,
more investigation into the role of Rb and p53 with respect to
centrosome amplification, chromosome instability, and tumorigenesis
is warranted.
To address experimentally the independent effects of p53 and Rb
inactivation with respect to centrosome abnormalities and genomic
instability, we used a previously established mouse model of skin
cancer (17). Our particular model system uses the human keratin 14
(K14) transcriptional promoter to express two HPV type 16 viral
oncogenes, E6 and E7, in the basal epithelial cells of the epidermis.
HPVs are DNA tumor viruses that cause benign tumors or warts in
human skin. A subset of HPVs, including HPV types 16 and 18, are
high-risk HPVs associated with cervical cancers (18, 19). In progress-
ing from a low-grade squamous intraepithelial lesion to a high-grade
squamous intraepithelial lesion, the high-risk HPV genomes are com-
monly integrated into the host’s genome, and the E6 and E7 onco-
genes become overexpressed (20). In binding to and degrading p53
and Rb, respectively, the E6 and E7 proteins confer transforming
abilities to the cell, but their contributions to carcinogenesis are
different. Molecular dissection of the respective pathways has shown
specifically that the expression of E7 contributes primarily to early
stages of carcinogenesis, leading to the formation of benign lesions. In
contrast, E6 affects primarily later stages of carcinogenesis, leading to
malignant conversion (20).
Previous observations of cervical (21), colorectal (22), breast (11),
and head and neck (23) tumors have revealed specific patterns of
Received 01/22/03; revised 10/21/03; accepted 11/6/03.
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Requests for reprints: Michael J. Difilippantonio, Section of Cancer Genomics,
National Institutes of Health, 50 South Drive, Room 1306, Bethesda, MD 20892-8010.
Phone: 301-435-3991; Fax: 301-402-1204; E-mail: difilipm@mail.nih.gov.
538
Research.
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