Homozygous familial defective apolipoprotein B-100: Enhanced removal of apolipoprotein E-containing VLDLs and decreased production of LDLs

Arteriosclerosis, Thrombosis, and Vascular Biology

Volumn 17, Issue 2, 1997, Pages 348-353

  Schaefer, Juergen R ^a   Scharnagl, Hubert ^b   Baumstark, Manfred W ^c   Schweer, Horst ^a   Zech, Loren A ^e   Seyberth, Hansjörg ^a   Winkler, Karl ^b   Steinmetz, Armin ^d   März, Winfried ^b,d  

^a PHILIPPS UNIVERSITY MARBURG   (Germany)

^b Division of Clinical Chemistry   (Germany)

^c UNIVERSITY OF FREIBURG   (Germany)

^d UNIVERSITY OF FREIBURG   (Germany)

^e NATIONAL HEART LUNG AND BLOOD INSTITUTE   (United States)

Author keywords

apolipoprotein B 100; atherosclerosis; genetic disease; hypercholesterolemia; stable isotope tracer kinetics

Indexed keywords

APOLIPOPROTEIN B; APOLIPOPROTEIN B100; APOLIPOPROTEIN E; LOW DENSITY LIPOPROTEIN; LOW DENSITY LIPOPROTEIN CHOLESTEROL; LOW DENSITY LIPOPROTEIN RECEPTOR; VERY LOW DENSITY LIPOPROTEIN;

ADULT; ARTICLE; CASE REPORT; FAMILIAL DISEASE; FAMILIAL HYPERCHOLESTEROLEMIA; GENE MUTATION; HUMAN; HUMAN CELL; HUMAN TISSUE; HYPOLIPOPROTEINEMIA; MALE; MEAN RESIDENCE TIME; PRIORITY JOURNAL; PROTEIN METABOLISM; RECEPTOR UPREGULATION;

EID: 0031026680     PISSN: 10795642     EISSN: None     Source Type: Journal    
DOI: 10.1161/01.ATV.17.2.348     Document Type: Article

References (8)

1. Myant NB. Cholesterol metabolism, LDL, and the LDL Receptor. San Diego, Calif: Academic Press Inc; 1990.
2. Young SG. Recent progress in understanding apolipoprotein B. Circulation. 1990;82:1574-1594.
3. Scott J. The molecular and cell biology of apolipoprotein B. Mol Biol Med. 1989;6:65-80.
4. Goldstein JL, Hobbs HH, Brown MS. Familial hypercholesterolemia. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Basis of Inherited Disease, 7th ed. New York, NY: McGraw Hill Inc; 1995;II:1981-2030.
5. Innerarity TL, Weisgraber KH, Arnold KS, Mahley RW, Krauss RM, Vega GL, Grundy SM. Familial defective apolipoprotein B-100: low density lipoproteins with abnormal receptor binding. Proc Natl Acad Sci U S A. 1987;84:6919-6923.
6. Weisgraber KH, Innerarity TL, Newhouse YM, Young SG, Arnold KS, Krauss RM, Vega GL, Grundy SM, Mahley RW. Familial defective apolipoprotein B-100: enhanced binding of monoclonal antibody MB47 to abnormal low density lipoproteins. Proc Natl Acad Sci U S A. 1988;85:9758-9762.
7. Soria LF, Ludwig EH, Clarke HRG, Vega GL, Grundy SM, McCarthy BJ. Association between a specific apolipoprotein B mutation and familial defective apolipoprotein B-100. Proc Natl Acad Sci USA. 1989;86:587-591.
8. Innerarity TL, Mahley RW, Weisgraber KH, Bersot TP, Krauss RM, Vega GL, Grundy SM, Friedl W, Davignon J, McCarthy BJ. Familial