Mechanisms for cellular cholesterol transport: Defects and human disease

被引:175
作者
Ikonen, Elina [1 ]
机构
[1] Univ Helsinki, Inst Biomed Anat, FIN-00014 Helsinki, Finland
关键词
D O I
10.1152/physrev.00022.2005
中图分类号
Q4 [生理学];
学科分类号
071003 ;
摘要
This review summarizes the mechanisms of cellular cholesterol transport and monogenic human diseases caused by defects in intracellular cholesterol processing. In addition, selected mouse models of disturbed cholesterol trafficking are discussed. Current pharmacological strategies to prevent atherosclerosis are largely based on altering cellular cholesterol balance and are introduced in this context. Finally, because of the organizing potential of cholesterol in membranes, disturbances in cellular cholesterol transport have implications for a wide variety of human diseases, of which selected examples are given.
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收藏
页码:1237 / 1261
页数:25
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共 278 条
[11]   Genetic and biochemical evidence that CESD and Wolman disease are distinguished by residual lysosomal acid lipase activity [J].
Aslanidis, C ;
Ries, S ;
Fehringer, P ;
Buchler, C ;
Klima, H ;
Schmitz, G .
GENOMICS, 1996, 33 (01) :85-93
[12]  
Assmann G, 1995, METABOLIC MOL BASES, P2563
[13]  
Austen B, 2002, J Nutr Health Aging, V6, P377
[14]   Effect of ezetimibe coadministered with atorvastatin in 628 patients with primary hypercholesterolemia - A prospective, randomized, double-blind trial [J].
Ballantyne, CM ;
Houri, J ;
Notarbartolo, A ;
Melani, L ;
Lipka, LJ ;
Suresh, R ;
Sun, S ;
LeBeaut, AP ;
Sager, PT ;
Veltri, EP .
CIRCULATION, 2003, 107 (19) :2409-2415
[15]   Transport of newly synthesized sterol to the sterol-enriched plasma membrane occurs via nonvesicular equilibration [J].
Baumann, NA ;
Sullivan, DP ;
Ohvo-Rekilä, H ;
Simonot, C ;
Pottekat, A ;
Klaassen, Z ;
Beh, CT ;
Menon, AK .
BIOCHEMISTRY, 2005, 44 (15) :5816-5826
[16]   Nuclear receptors in lipid metabolism: Targeting the heart of dyslipidemia [J].
Beaven, SW ;
Tontonoz, P .
ANNUAL REVIEW OF MEDICINE, 2006, 57 :313-329
[17]   Accumulation of dietary cholesterol in sitosterolemia caused by mutations in adjacent ABC transporters [J].
Berge, KE ;
Tian, H ;
Graf, GA ;
Yu, LQ ;
Grishin, NV ;
Schultz, J ;
Kwiterovich, P ;
Shan, B ;
Barnes, R ;
Hobbs, HH .
SCIENCE, 2000, 290 (5497) :1771-1775
[18]  
Berriot-Varoqueaux N, 2000, ANN ENDOCRINOL-PARIS, V61, P125
[19]   BETA-SITOSTEROLEMIA AND XANTHOMATOSIS - NEWLY DESCRIBED LIPID STORAGE DISEASE IN SISTERS [J].
BHATTACHARYYA, AK ;
CONNOR, WE .
JOURNAL OF CLINICAL INVESTIGATION, 1974, 53 (04) :1033-1043
[20]   Genes involved in initial steps of bile acid synthesis [J].
Björkhem, I ;
Eggertsen, G .
CURRENT OPINION IN LIPIDOLOGY, 2001, 12 (02) :97-103