Remnants of lipoproteins of intestinal and hepatic origin in familial dysbetalipoproteinemia.
Abstract
We used the low molecular weight form of apolipoprotein B (B-48) as a marker for the identification of remnant particles formed from chylomicrons in the plasma of patients with familial dysbetalipoproteinemia. In the serum of patients fasted 14 hours, the d less than 1.006 g/cm3 lipoproteins of prebeta mobility, separated by starch block electrophoresis, contained only the primary hepatogenous species of apolipoprotein B (B-100), and their lipid composition resembled that of normal prebeta very low density lipoproteins. In contrast, the fraction with beta mobility contained both the B-48 and B-100 proteins; the B-48 protein was found primarily among the largest particles. All fractions of beta mobility were greatly enriched with cholesteryl esters. The beta fraction thus contains remnant particles which appear to originate both from chylomicrons and hepatogenous very low density lipoproteins. It appears that these remnant particles share a common removal mechanism which is impaired in familial dysbetalipoproteinemia.
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Defective binding of apoE mutants to lipoprotein receptors causes impaired clearance of chylomicron and very low density lipoprotein remnants (β-VLDL), which accumulate in plasma and cause hypercholesterolemia and hypertriglyceridemia.
“This defective binding causes impaired clearance of chylomicron and very low density lipoprotein remnants (β-VLDL) (5, 6), which accumulate in the plasma and cause both hypercholesterolemia and hypertriglyceridemia.”
From Apolipoprotein E3-Leiden contains a seven-amino acid insertion that is a tandem repeat of residues 121–127 (1989) · cites this paper as [6]
Cites
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Self-citation
- Authors
- Kane, J. P., Chen, G. C., Hamilton, R. L., Hardman, D. A., Malloy, M. J., & Havel, R. J.
- ORCID
- Venue
- Arteriosclerosis: An Official Journal of the American Heart Association, Inc.
- Published
- 1983-01
- Volume
- 3
- Issue
- 1
- Pages
- 47-56
- ISSN
- 0276-5047 (ISSN-L)
- OpenAlex
- W2128590563
- Cited by
- 106
- References
- 2
- Citations by year
- 2018: 1 · 2014: 2 · 2013: 2 · 2012: 1
- FWCI
- 9.30
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Arteriosclerosis An Official Journal of the American Heart Association Inc (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Lipid metabolism and disorders
- Health Sciences › Medicine › Surgery › Lipoproteins and Cardiovascular Health
- Health Sciences › Medicine › Surgery › Cholesterol and Lipid Metabolism
- Keywords
- familial dysbetalipoproteinemia, remnant lipoproteins, apolipoprotein B-48, apolipoprotein B-100, chylomicrons, VLDL, cholesterol esters, lipoprotein metabolism, lipoprotein electrophoresis
- MeSH terms
- Adult, Amino Acids, Apolipoproteins, Apolipoproteins B, Chylomicrons, Circular Dichroism, Electrophoresis, Female, Humans, Hyperlipoproteinemia Type III, Intestinal Mucosa, Lipoproteins, LDL, Lipoproteins, VLDL, Liver, Male
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