Vol. 3, 2025-2032, November 1997 Clinical Cancer Research 2025
Altered Methylation of the Human MDR1 Promoter Is Associated
with Acquired Multidrug Resistance1
Phillip Kantharidis, Assam El-Osta,
Michelle deSilva, Dominic M. P. Wall, Xiu F. Hu,
Alison Slater, Gabriella Nadalin, John D. Parkin,
and John R. Zalcberg2
Departments of Medical Oncology and Haematology, Austin
Repatriation Medical Centre, West Heidelberg 308 1 . Victoria,
Australia [D. M. P. W., J. D. P]: and Trescowthick Research Labs
[P. K.. A. E-O., M. d., X. F. H., A. S., G. N.] and Division of
Haematology and Medical Oncology [J. R. Z.], Peter MacCallum
Institute, East Melbourne 3002, Victoria, Australia
ABSTRACT
One of the most important forms of drug resistance in
acute myeboid leukemia is the multidrug resistance (MDR)
phenotype, which is characterized by the expression of the
MDRJ gene product, P-glycoprotein. Although a number of
factors affect MDR1 gene expression, the genetic events that
“switch on” the human MDRJ gene in tumor cells that were
previously P-glycoprotein negative have remained elusive.
Here, we report evidence that the methybation status of the
human MDR1 promoter may serve as a basis for this
“switch.” Based on Southern analysis using methylation-
sensitive and methylation-insensitive restriction enzymes, a
tight correlation was found between MDR phenotype and
demethylation of the 5’ region of the MDRJ gene in a human
T cell leukemia cell line. Similar results were obtained from
the analysis of P-glycoprotein-positive and P-glycoprotein-
negative samples of chronic lymphocytic leukemia. Treat-
ment of the cell lines with the demethybating agent 5’-
azadeoxycytidine altered the methybation pattern of the
MDR1 promoter in P-glycoprotein.negative cells to resem-
ble that of P-gbycoprotein-positive cells and activated the
promoter such that MDR1 mRNA was now detectable.
Treatment also resulted in an increased resistance to epiru-
bicin and decreased daunomycin accumulation, both of
which were reversible by verapamil, a characteristic of the
classical MDR phenotype in cells expressing P-gbycoprotein.
These results suggest that the MDR phenotype may be
Received I 1/13/96; revised 4/25/97; accepted 7/3/97.
The costs of publication of this article were defrayed in part by the
payment of page charges. This article must therefore be hereby marked
advertisement in accordance with I8 U.S.C. Section 1734 solely to
indicate this fact.
1 This work was supported by the Anti-Cancer Council of Victoria and
Department of Veterans’ Affairs (Canberra, ACT. Australia) and the Sir
Edward Dunlop Medical Research Foundation (Melbourne, Victoria,
Australia).
2 To whom requests for reprints should be addressed, at Division of
Haematology and Medical Oncology. Peter MacCallum Cancer Insti-
tute, East Melbourne 3000, Victoria, Australia. Phone: (613) 9656 1190;
Fax: (613) 9656 1408; E-mail: zalcberg@petermac.unimelb.edu.au.
acquired as a result of changes in methylation of the MDR1
promoter.
INTRODUCTION
Drug resistance remains a major obstacle in the successful
treatment of human tumors by cytotoxic agents. Of the several
forms of drug resistance described, the most extensively studied
is classical MDR,3 which is characterized by the overexpression
of the MDRJ gene product, Pgp; partial reversal of resistance by
several modulators, including verapamil and cyclosporin A; and
cross-resistance to a variety of naturally occurring cytotoxic
agents (1 ). This plasma membrane glycoprotein functions as an
energy-dependent drug efflux transporter, resulting in bower
intracellular levels of a wide variety of chemotherapeutic agents
that are substrates for Pgp (1). Despite the difficulties in suc-
cessfully modulating this phenotype clinically (2), the expres-
sion of Pgp is of prognostic significance in various hematolog-
ical malignancies, including acute myeboid leukemia. In a series
of unrelated studies, Pgp expression represented an adverse
prognostic factor that independently predicted for survival, re-
sponse rate, and duration of response (reviewed in Ref. 2).
In human cancers in which the corresponding normal tissue
expresses Pgp, the resulting tumors also appear to express this
protein. These include carcinomas of the colon, liver, pancreas,
and kidney (1). However, in drug-sensitive tumors, the acqui-
sition of MDR during the course of chemotherapy is thought to
be due to the selection of resistant mutants in the tumor cell
population by drugs known to be substrates for Pgp. This model
fits with the mutation-selection hypothesis for drug resistance in
cancer (3), in which genetic changes in a small number of
drug-sensitive cells ultimately lead to the development of re-
sistance via a process of selection. This scenario is thought to be
particularly relevant in hematological malignancies.
Although the regulation of Pgp expression has been studied
in several cell types and much has been learned about factors
regulating its expression (4-8), the genetic events that switch
on Pgp expression in Pgp-negative cells remain poorly defined.
No mutations in the sequence of the MDR 1 promoter have been
identified to account for the activation of the MDR 1 promoter in
cells that express Pgp. Although point mutations have been
reported in a number of osteosarcomas at nucleotides 103 and
107 downstream of the MDR1 transcription initiation site, they
were found in untreated tumors, and their significance remains
to be determined (9).
Attempts have been made to identify transcription factors
that may be altered or present in higher or lower levels in
Pgp-positive cells compared to Pgp-negative cells. One report
3 The abbreviations used are: MDR, multidrug resistance: Pgp. P-gly-
coprotein: CLL. chronic lymphocytic leukemia: azadC, 5 ‘-azadeoxycy-
tidine; RT-PCR, reverse transcription-PCR.
Research.
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