Orso, E and Broccardo, C and Kaminski, WE and Bottcher, A and Liebisch, G and Drobnik, W and Gotz, A and Chambenoit, O and Diederich, W and Langmann, T and Spruss, T and Luciani, MF and Rothe, G and Lackner, KJ and Chimini, G and Schmitz, G (2000) Transport of lipids from Golgi to plasma membrane is defective in Tangier disease patients and Abc1-deficient mice. NATURE GENETICS, 24 (2). pp. 192-196. ISSN 1061-4036,
Full text not available from this repository.Abstract
Mutations in the gene encoding ATP-binding cassette transporter 1 (ABC1) have been reported in Tangier disease(1-3) (TD), an autosomal recessive disorder that is characterized by almost complete absence of plasma high-density lipoprotein (HDL), deposition of cholesteryl esters in the reticulo-endothelial system(4) (RES) and aberrant cellular lipid trafficking(5-12). We demonstrate here that mice with a targeted inactivation of Abc1 display morphologic abnormalities and perturbations in their lipoprotein metabolism concordant with TD. ABC1 is expressed on the plasma membrane and the Golgi complex, mediates apo-Al associated export of cholesterol and phospholipids from the cell, and is regulated by cholesterol flux. Structural and functional abnormalities in caveolar processing and the trans-Golgi secretory pathway of cells lacking functional ABC1 indicate that lipid export processes involving vesicular budding between the Golgi and the plasma membrane are severely disturbed.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | FREE-CHOLESTEROL; A-I; LIPOPROTEINS; MACROPHAGES; METABOLISM; CAVEOLAE; EFFLUX; CELLS; |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Klinische Chemie und Laboratoriumsmedizin |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 21 May 2021 07:48 |
| Last Modified: | 21 May 2021 07:48 |
| URI: | https://pred.uni-regensburg.de/id/eprint/42865 |
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