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Accumulation of Dietary Cholesterol in Sitosterolemia Caused by Mutations in Adjacent ABC Transporters
Knut E. Berge,1*Hui Tian,3*Gregory
A. Graf,1Liqing Yu,1Nick V. Grishin,2Joshua Schultz,3Peter Kwiterovich,4Bei Shan,3Robert Barnes,1Helen H. Hobbs1
In healthy individuals, acute changes in cholesterol
intake produce modest changes in plasma cholesterol levels. A strikingexception occurs in sitosterolemia, an autosomal recessive disordercharacterized by increased intestinal absorption and decreasedbiliary
excretion of dietary sterols, hypercholesterolemia, andpremature
coronary atherosclerosis. We identified seven differentmutations in
two adjacent, oppositely oriented genes that encodenew members of the
adenosine triphosphate (ATP)-binding cassette(ABC)
transporter family (six mutations in ABCG8 and one in
ABCG5)in nine patients with sitosterolemia. The two genes
are expressedat highest levels in liver and intestine and, in mice,
cholesterolfeeding up-regulates expressions of both genes. These data
suggestthat ABCG5 and ABCG8 normally cooperate to limit intestinal
absorptionand to promote biliary excretion of sterols, and that
mutatedforms of these transporters predispose to sterol accumulationand atherosclerosis.
1 Department of Molecular Genetics and
McDermott Center for Human Growth and Development and
2 Howard Hughes Medical Institute and Department of
Biochemistry, University of Texas Southwestern Medical Center at
Dallas, 5323 Harry Hines Boulevard, Dallas, TX 75390-9046, USA.
3 Tularik Inc., Two Corporate Drive, South San
Francisco, CA 94080, USA.
4 Department of
Pediatrics, Johns Hopkins University, Baltimore, MD 21205, USA.
*
These authors contributed equally to the work.
To whom correspondence should be addressed:
Helen.Hobbs{at}UTSouthwestern.edu
The editors suggest the following Related Resources on Science sites:
In Science Magazine
PERSPECTIVES
Hooman Allayee, Bryan A. Laffitte, and Aldons J. Lusis (1 December 2000) Science290 (5497), 1709.
[DOI: 10.1126/science.290.5497.1709] |Summary »|Full Text »
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Aminopeptidase N (CD13) Is a Molecular Target of the Cholesterol Absorption Inhibitor Ezetimibe in the Enterocyte Brush Border Membrane.
W. Kramer, F. Girbig, D. Corsiero, A. Pfenninger, W. Frick, G. Jahne, M. Rhein, W. Wendler, F. Lottspeich, E. O. Hochleitner, et al. (2005)
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280, 1306-1320
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Genetics of Variation in HDL Cholesterol in Humans and Mice.
Megalin and cubilin expression in gallbladder epithelium and regulation by bile acids.
B. Erranz, J. F. Miquel, W. S. Argraves, J. L. Barth, F. Pimentel, and M.-P. Marzolo (2004)
J. Lipid Res.
45, 2185-2198
|Abstract »|Full Text »|PDF »
Phenotypic heterogeneity of sitosterolemia.
J. Wang, T. Joy, D. Mymin, J. Frohlich, and R. A. Hegele (2004)
J. Lipid Res.
45, 2361-2367
|Abstract »|Full Text »|PDF »
No Association Between Plasma Levels of Plant Sterols and Atherosclerosis in Mice and Men.
K. R. Wilund, L. Yu, F. Xu, G. L. Vega, S. M. Grundy, J. C. Cohen, and H. H. Hobbs (2004)
Arterioscler Thromb Vasc Biol
24, 2326-2332
|Abstract »|Full Text »|PDF »
