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Titolo:
Transport of lipids from Golgi to plasma membrane is defective in Tangier disease patients and Abc1-deficient mice
Autore:
Orso, E; Broccardo, C; Kaminski, WE; Bottcher, A; Liebisch, G; Drobnik, W; Gotz, A; Chambenoit, O; Diederich, W; Langmann, T; Spruss, T; Luciani, MF; Rothe, G; Lackner, KJ; Chimini, G; Schmitz, G;
Indirizzi:
Univ Regensburg, Inst Clin Chem & Lab Med, D-93042 Regensburg, Germany Univ Regensburg Regensburg Germany D-93042 , D-93042 Regensburg, Germany CNRS Marseille Luminy, INSERM, Ctr Immunol, Marseille 9, France CNRS Marseille Luminy Marseille France 9 tr Immunol, Marseille 9, France Univ Regensburg, Lab Anim Facil, D-8400 Regensburg, Germany Univ Regensburg Regensburg Germany D-8400 il, D-8400 Regensburg, Germany
Titolo Testata:
NATURE GENETICS
fascicolo: 2, volume: 24, anno: 2000,
pagine: 192 - 196
SICI:
1061-4036(200002)24:2<192:TOLFGT>2.0.ZU;2-4
Fonte:
ISI
Lingua:
ENG
Soggetto:
FREE-CHOLESTEROL; A-I; LIPOPROTEINS; MACROPHAGES; METABOLISM; CAVEOLAE; EFFLUX; CELLS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
30
Recensione:
Indirizzi per estratti:
Indirizzo: Schmitz, G Univ Regensburg, Inst Clin Chem & Lab Med, Franz Josef Str Allee 11, D-93042 Regensburg, Germany Univ Regensburg Franz Josef Str Allee 11 Regensburg Germany D-93042
Citazione:
E. Orso et al., "Transport of lipids from Golgi to plasma membrane is defective in Tangier disease patients and Abc1-deficient mice", NAT GENET, 24(2), 2000, pp. 192-196

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 functionalabnormalities in caveolar processing and the trans-Golgi secretory pathwayof cells lacking functional ABC1 indicate that lipid export processes involving vesicular budding between the Golgi and the plasma membrane are severely disturbed.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/12/20 alle ore 18:10:20